Pulmonary arterial hypertension (PAH) is an increasingly recognized complication of HIV disease associated with significant morbidity and mortality. The prevalence of PAH is estimated to be 0.5% in HIV-infected patients compared to the 0.02% in non HIV-infected patients. A beneficial effect of antiretroviral therapy on the course of HIV-related PAH has not been clearly established. Bosentan, a dual endothelin-1 receptor antagonist, may be an effective approach to treatment of PAH in HIV-infected patients. We report a first case of a successful therapy with bosentan in an HIV-infected patient treated with fosamprenavir/ritonavir based-regimen.