Pharming and Swedish Orphan Biovitrum Amend and Extend Ruconest Distribution Agreement

Pharming to Directly Commercialise Ruconest in Austria Germany and Netherlands

Biotech company Pharming Group NV (“Pharming” or “the Company”) (EURONEXT: PHARM) and Swedish Orphan Biovitrum AB (publ) or “Sobi” (SS: SOBI) today announced the amendment and extension of the Ruconest® distribution agreement between Pharming and Sobi. Effective immediately Pharming will focus on direct commercialization in Austria Germany and Netherlands and Sobi will extend its Ruconest® sales territory with the addition of Azerbaijan Belarus Georgia Kazakhstan Russia Serbia and Ukraine.

Pharming has recently started the hiring of a small European team of experienced HAE commercialisation and medical affairs specialists to take-over the direct commercialization activities from Sobi for Ruconest®in Austria Germany and Netherlands. To ensure a seamless hand- over in particular to guarantee the continuous availability of Ruconest® to patients and the drug safety monitoring and reporting of Ruconest® Sobi will continue to deliver Ruconest® as before and will continue the drug safety monitoring and reporting during the remaining months of 2014.

Sijmen de Vries Pharming CEO commented “This step forward into direct commercialization of Ruconest® became possible as result of our recently improved balance sheet and opens up new opportunities for Pharming to not only grow revenues but also as those revenues build to put in place the right size of specialist commercial infrastructure which could over time be leveraged through the marketing of other products. This move is expected to almost immediately have a positive impact on our financial performance as we will be able to improve the margin from sales in the EU territories with every vial of Ruconest® sold in Austria Germany and Netherlands. In addition the extension of the Sobi territory will enable Sobi to continue growing Ruconest® revenues further.

About Pharming Group NV:

Pharming Group NV is developing innovative products for the treatment of unmet medical needs. Ruconest® (conestat alfa) is a recombinant human C1 esterase inhibitor approved for the treatment of angioedema attacks in patients with HAE in the USA Israel all 27 EU countries plus Norway Iceland and Liechtenstein. Ruconest® is commercialized by Pharming in Austria Germany and Netherlands. Ruconest® is distributed by Swedish Orphan Biovitrum AB (publ) (SS: SOBI) in the other EU countries and in Azerbaijan Belarus Georgia Iceland Kazakhstan Liechtenstein Norway Russia Serbia and Ukraine.

Ruconest® is partnered with Salix Pharmaceuticals Inc. (NASDAQ: SLXP) in North America.

The product is also being investigated in a randomized Phase II clinical trial for prophylaxis of HAE and evaluated for various additional follow-on indications. Pharming has a unique GMP compliant validated platform for the production of recombinant human proteins that has proven capable of producing industrial volumes of high quality recombinant human protein in a more economical way compared to current cell based technologies. In July 2013 the platform was partnered with Shanghai Institute for Pharmaceutical Industry (SIPI) a Sinopharm Company for joint global development of new products. Pre- clinical development and manufacturing will take place at SIPI and are funded by SIPI. Pharming and SIPI initially plan to utilize this platform for the development of rh-FVIII for the treatment of Haemophilia-A. Additional information is available on the Pharming website;