Human immunodeficiency virus (HIV) attacks the immune system and can cause acquired immunodeficiency syndrome. Thankfully, antiretroviral therapy is highly effective, and people infected with HIV can live long lives with proper adherence to therapy. One of the important noninfectious complications of HIV is the development of pulmonary arterial hypertension (PAH). This review will cover the epidemiology, pathogenesis, and clinical characteristics of HIV-associated PAH. It will also cover the current knowledge on use of vasodilator therapy in this population.