Pericarditis in patients with human immunodeficiency virus (HIV) infection may be asymptomatic or symptomatic, but is sometimes overlooked because of signs and symptoms of other organ system diseases. A case report of cardiac tamponade in a patient with acquired immunodeficiency syndrome (AIDS) is presented. To determine the incidence and causes of pericardial disease in patients with HIV infection, a review of the literature was conducted. Fifteen autopsy and echocardiographic series involving 1139 patients with HIV infection reveal that the average incidence of pericardial disease is 21%. Most cases are asymptomatic and without an identifiable cause. However, in those that are symptomatic, about two thirds are caused by infection or neoplasm; one third have an undetermined etiology. In the 66 published cases of cardiac tamponade in patients with HIV infection, 26% are caused by tuberculosis, 17% are purulent, and 8% are caused by Mycobacterium avium-intracellulare (MAI). Lymphoma and Kaposi's sarcoma are each responsible for 5% of the effusions. Less frequent causes of tamponade are Cryptococcus neoformans, cytomegalovirus, and Mycobacterium kansasii. Small asymptomatic pericardial effusions in patients with HIV infection do not require diagnostic evaluation. However, large symptomatic pericardial effusions should be investigated, because two thirds are caused by potentially treatable infections or neoplasms.