In recent years, much more insight is given to the pathogenic role of HIV and to the clinical manifestations of HIV-related pulmonary hypertension (HRPH), that currently represents one of the most severe events during the HIV disease. HRPH occurs in early and late stages of HIV infection and does not seem to be related to the degree of immune deficiency. Many of the symptoms in HRPH result from right ventricular dysfunction: the first clinical manifestation is effort intolerance and exertional dyspnoea that will progress to the point of breathlessness at rest. Echocardiography has been proved to be an extremely useful tool for the diagnosis of HRPH, and Doppler echocardiography may be used to estimate systolic pulmonary artery pressure, and to monitor the effects of therapy. Assessment of hemodynamic measures by catheterization remains, however, the best test for evaluating the response to therapy. Cardiac catheterization is mandatory to characterize the disease and exclude an underlying cardiac shunt as etiology. Vasodilators have been extensively used in the treatment of pulmonary hypertension, since vasoconstriction is a determinant characteristic of this disease. More recently, more effective therapies for pulmonary arterial hypertension have been available, including prostanoids, endothelin-receptor antagonists, and phosphodiesterase-5 inhibitors, allowing an amelioration of symptoms and a better prognosis. However, HRPH remains a progressive disease for which treatment is often unsatisfactory and there is no cure. As new efficient antiretroviral treatment will be introduced, clinicians should expect to encounter an increasing number of cases of pulmonary hypertension in HIV-infected patients in the future.