Very Strong Performancein the First Half of 2016 Enhanced by Successful Uptravi US Launch

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Media Release

21 July 2016

Very strong performancein the first half of 2016–enhanced by successful Uptravi US launch

ALLSCHWIL/BASEL, SWITZERLAND – 21 July 2016– Actelion Ltd (SIX: ATLN) today announced its results for the first six months of 2016.

OPERATINGHIGHLIGHTS

• Excellent Opsumit® (macitentan) trajectory andnew patient referralssustained
• Continued outstanding US launchmomentum for Uptravi® (selexipag), Q2 launches in Germany, Canada (private market) and France (cohort ATU)
• Initiation of Phase III study to evaluate the efficacy and safety of long-term use ofmacitentan (Opsumit) for the treatment ofchildren with PAH
• Initiation of Phase II program with new dual orexin receptor antagonist for the treatment of insomnia

FINANCIAL HIGHLIGHTS

• Sales of CHF 1,179million(+13% at CER) – portfolio transformation well underway
• Opsumitsales grow to CHF 378million
• Uptravi sales of CHF 90 million
• US GAAP operating income of CHF 412 million (+12% at CER)
• Core operating income of CHF 499million(+11% at CER)
• 2016 financial guidanceupgrade: core operating income growth in the low-teen percentage range,at constant exchange rates and barring unforeseen events

(1)CER percentage changes are calculated by reconsolidating both the H1 2015andH1 2016 results at constant currencies (the average monthly exchange rates for H1 2015).

(2)Actelion continues to measure, report and issue guidance on its core operating performance, which management believes more accurately reflects the underlying business performance. The Group believes that these non-GAAP financial measurements provide useful supplementary information to investors. These non-GAAP measures are reported in addition to, not as a substitute for, US GAAP financial performance.

Jean-Paul Clozel, MD, Chief Executive Officer,commented: “We have had an excellent first half year, mainly driven by the strong performance of both Opsumit and Uptravi. We have also made significant progress with our development pipeline byinitiatinga Phase III long-term study with macitentan for the treatment ofchildren with PAH anda Phase II program with our new dual orexin receptor antagonist. All in all, the transformation of the company is well underway and I am very pleased with our achievements.What’s more,I am confident that we can sustain this momentum moving forward.”

Otto Schwarz, Chief Operating Officer, commented: “The strong uptake of Uptravi is the result of the robust clinical data generated with thisinnovative drug,combined with our strong commercial capabilities to launch specialty therapies. Sales reached 90 million Swiss francs for the first six months of the year with about 1,150 patients benefiting from Uptravi at the end of June.Along with the continued strong Opsumit launch momentum and the Veletri growth, the transformation of our PAH portfolio is progressing well, with almost half of the second quarter 2016 sales stemming from our new outcome-based therapies.”

André C. Muller, Chief Financial Officer, commented:“The balance of sales growth with disciplined R&D investment in the company’s future has resulted in double-digit US GAAP and core operating income growth.Based on this excellent first half year, along with more clarity on a number of factors, including the strong Opsumit and Uptravi launch dynamics, stable pricing in Japan for Opsumit and Tracleer, and the unlikely entry of generic competition for Tracleer in the US in 2016, the company has increased the outlook for the full year. Barring unforeseen events, we now expect 2016 core operating income growth in the low-teen percentage range at constant exchange rates.”

SALES UPDATE

Actelion delivered an outstanding first half 2016, driven bya very strong Uptravi launchin the US and the continued successful uptake of Opsumit. The excellent performance of the company’s outcome-based PAH portfolio is the result of consistently strong referralsof new PAH patients across markets, as well asan increase in the number of patients benefitting fromdouble and triple combination PAH therapy.

In the US, sales increased by 25% at CER, driven by the strong Uptravi launch, continued Opsumit momentum and ERA market share gains. European sales were 1% lower at CER,despite increased Opsumit uptake and Tracleer use in the digital ulcer indication,due to continued pricing pressure and market erosion from generics, particularly in Spain.Sales in Japan increased by 20% at CER, mostly driven by very strong sales ofOpsumit (launched in June 2015), Veletri and Zavesca (Japanese trade name Brazaves).

Comparing average exchange rates for the first six months of 2016 to the first six months of 2015, the Swiss franc weakened, mostly against the US dollar, euro and Japanese yen, resulting in a positive currency variance of 39 million Swiss francs.

Sales by product – H1 2016

Sales by product – Q2 2016

Sales by region – H1 2016

*Europe = EU28 and Switzerland

Sales by region – Q2 2016

*Europe = EU28 and Switzerland

PAH FRANCHISE

Opsumit®

Sales of Opsumit (macitentan) amounted to 378 million Swiss francs for the first six months of 2016, an increase of 76% at CER compared to the first six months of 2015. This increase continues to be driven by the uptake trajectory with commercial availability in over 30 countries. The strong increase in patients benefitting from Opsumitis driven by referral of treatment-naïve patients together with increased early combination with PDE-5 inhibitors, and some switching from Tracleer, notably in Japan.

Uptravi®

Sales of Uptravi (selexipag) amounted to 90 million Swiss francs for the first six months of 2016. Of this total amount, approximately 30 million Swiss francs can be attributed to the build-up of the US launch inventory as 10 different presentations of the drug across the various doses were made available. Uptravi has been fully launched in the US since 4 January 2016 and in Germany since 15 June 2016.

During the second quarter 2016, Uptravi also became available in France under a cohort ATU and in Canada for the private market segment. At the end of June, about 1,150 patients were using this outcome-based, oral selective IP receptor agonist.

Tracleer®

Sales of Tracleer (bosentan) amounted to 546 million Swiss francs for the first six months of 2016, a decrease of 18% at CER compared to the first six months of 2015. This decrease is mostly a consequence of lower usein countries where Opsumit is available, due to lower referralof new patients as well as switches to Opsumit. Underlying volumes decreased globally by 16%. Tracleer sales were further impacted by increased generic bosentan competition, notably in Spain, continued pricing pressure in Europe, and order phasing/inventory adjustment in the US.

Positively, Tracleer sales were supported by the digital ulcer indication in Europe and Japan and continued solid demand in markets where Opsumit is not yet available.

Following the Pediatric Investigation Plan (PIP) compliance statement from the European Committee for Medicinal Products for Human Use (CHMP), applications for extension of the Supplementary Protection Certificate (SPC) were filed in 19 EU countries and have been granted in 15 of those,with 4 still pending.

Veletri®

Salesof Veletri (epoprostenol for injection) amounted to 48 million Swiss francs for the first six months of 2016, an increase of 19% at CER, compared to the first six months of 2015. This increase was mostly driven by increased market penetration, successful launches in additional markets, and continued growth in Japan (where it is marketed as Epoprostenol “ACT”). In March 2016, Actelion Japan was notified of an average 12% price cut for Veletri, effective March 1, 2016. At the end of June 2016, Veletri was available in 15 countries globally.

Ventavis®

Sales of Ventavis (iloprost) amounted to 43 million Swiss francs for the first six months of 2016, a decrease of 27% at CER, compared to the first six months of 2015. This is due to the competitive environment, including the availability of Uptravi. Underlying units decreased by 34%.

SPECIALTY PRODUCTS

Valchlor®

Sales of Valchlor (mechlorethamine) amounted to 18 million Swiss francs for the first six months of 2016, an increase of 43% at CER, compared to the first six months of 2015. In the US, the company is continuing its efforts to establish Valchlor as an option in the treatment algorithm for early-stage mycosis fungoides, a type of Cutaneous T-Cell Lymphoma (MF-CTCL).

In France, patients benefited from the drug under a temporary nominative authorization for use (“ATU”) program initiated during the second half of 2014. The regulatory dossier is currently under review with the European Medicines Agency (under the trade name Ledaga®).

Zavesca®

Sales of Zavesca (miglustat) amounted to 52 million Swiss francs for the first six months of 2016, an increase of 15% at CER compared to the first six months of 2015.Sales in the US were strong due to a relatively low prior year base as a consequence of last year’s inventory adjustment. The global number of patients receiving therapy grew by 5% compared to the first half of 2015, driven by a 14% increase in Niemann-Pick type C demand.

In Europe, sales decreased by 2% mainly due to the launch of generic miglustat (approved for the type 1 Gaucher disease indication only), which has become commercially available in Spain, Sweden and the Czech Republic. Sales in Japan were 19% higher, driven by increased patient demand in the Niemann-Pick type C indication.

PIPELINE

Actelion’s pipeline has made significant progress, with macitentan (Opsumit) enteringinto Phase III for the treatment ofchildren with PAH, andthe advancement of the new dual orexin receptor antagonist into Phase II developmentfor the treatment of insomnia. In addition, inclusion of participantsin the ongoing Phase III studies for cadazolid and ponesimod is progressing well and is on target to be completed by the end of 2016. The MERIT study ofmacitentan for the treatment of patients with chronic thromboembolic pulmonary hypertension (CTEPH) has completed enrollment and is on schedule to deliver results by the end of the year.

RESULTS DAY CENTER

Investor community: To make your job easier, we provide links to all relevant documentation, such as a full financial review, reconciliation US-GAAP to Core results and geographical breakdown by product, from the Results Day Center on our corporate website:

HALF YEAR REPORT

Full details on the progress made during the first six months of 2016 are available in Actelion's 2016 Half Year Report, available from

UPCOMING CLINICAL UPDATES

• Lucerastat future development
• Cardiovascular pipeline update with MERIT results
• Cadazolid Phase III program update
• Ponesimod Phase III program update

UPCOMING FINANCIAL UPDATES

• 9M 2016 Financial Results reporting on 20 October 2016
• FY 2016 Financial Results reporting on 14 February 2017

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NOTES TO THE EDITOR

ABOUT ACTELION LTD.

Actelion Ltd. is a leading biopharmaceutical company focused on the discovery, development and commercialization of innovative drugs for diseases with significant unmet medical needs.

Actelion is a leader in the field of pulmonary arterial hypertension (PAH). Our portfolio of PAH treatments covers the spectrum of disease, from WHO Functional Class (FC) II through to FC IV, with oral, inhaled and intravenous medications. Although not available in all countries, Actelion has treatments approved by health authorities for a number of specialist diseases including Type 1 Gaucher disease, Niemann-Pick type C disease, Digital Ulcers in patients suffering from systemic sclerosis, and mycosis fungoides type cutaneous T-cell lymphoma.

Founded in late 1997, with now over 2,500 dedicated professionals covering all key markets around the world including Europe, the US, Japan, China, Russia and Mexico, Actelion has its corporate headquarters in Allschwil / Basel, Switzerland.

Actelion shares are traded on the SIX Swiss Exchange (ticker symbol: ATLN) as part of the Swiss blue-chip index SMI (Swiss Market Index SMI®). All trademarks are legally protected.

For further information please contact:

Andrew Weiss

Senior Vice President, Head of Investor Relations & Corporate Communications

Actelion Pharmaceuticals Ltd, Gewerbestrasse 16, CH-4123 Allschwil

+41 61 565 62 62

- First Half 2016 Financial Results -