Pulmonary arterial hypertension (PAH) is a rare but severe disease that results from chronic obstruction of small pulmonary arteries, leading to right ventricular failure and, ultimately, death. One established risk factor for the development of PAH is HIV infection. In comparison with the incidence of idiopathic PAH in the general population (1-2 per million), HIV-infected patients have a 2500-fold increased risk of developing PAH. The presence of PAH is an independent risk factor for mortality in patients with HIV infection, and in most cases death is causally related to PAH rather than to other complications of HIV infection. This article will focus on HIV-PAH with special considerations to epidemiology, pathogenesis, clinical presentation, diagnostic approach and available treatments.