Pulmonary hypertension is a severe life-limitating disease often affecting younger patients. The connection between HIV infection and the development of pulmonary hypertension is well documented. The underlying pathobiology still remains unclear. Given that the prognosis of HIV infection has been improved by highly active antiretroviral therapy (HAART), severe pulmonary hypertension is becoming a life-limiting factor.HIV patients suffering from exercise-induced dyspnea should be tested for pulmonary hypertension, if other pulmonary or cardiac disorders (e. g., restrictive or obstructive ventilation disorders, pneumonia, coronary heart disease) can be excluded. The incidence of pulmonary hypertension is 1,000 times higher in HIV patients as compared to the general population. Estimated numbers of unreported cases are not included.A suspected diagnosis of pulmonary hypertension can be substantiated by noninvasive diagnostic methods (e. g., echocardiography), however, right heart catheterization remains the diagnostic gold standard. As new therapeutic options with prostanoids, endothelin antagonists, and phosphodiesterase-5 inhibitors are now available, early and accurate diagnosis is essential.