As advances in early diagnosis and aggressive therapy, as well as better supportive care, become available to a larger number of patients with HIV infection, survival is being prolonged, and more patients are experiencing cardiac abnormalities. The most common cardiac manifestations of HIV disease are dilated cardiomyopathy, myocarditis, pericardial effusion, endocarditis, pulmonary hypertension, HIV-associated malignant neoplasms, and drug-related cardiotoxicity. The introduction of highly active antiretroviral therapy (HAART) regimens has substantially modified the course of HIV disease by lengthening survival and improving quality of life of HIV-infected patients. However, early data have raised concerns about HAART being associated with an increase in peripheral and coronary arterial disease. This review discusses the principal HIV-associated cardiovascular manifestations and emphasizes new knowledge about their prevalence, pathogenesis, and treatment.