Antiretroviral therapy has greatly increased longevity for individuals with human immunodeficiency virus (HIV) infection. About 0.5% of patients with HIV infection develop moderate to severe pulmonary arterial hypertension, which is several thousand times higher than the incidence of idiopathic pulmonary arterial hypertension. As more than 30 million individuals are chronically infected, HIV infection could soon become one of the most common causes of pulmonary arterial hypertension worldwide. Pulmonary arterial hypertension is a relentlessly progressive disease leading to right heart failure and death. In this article the available data on epidemiology, hemodynamics, mechanisms, and therapeutic strategies for HIV-associated pulmonary arterial hypertension are reviewed.