With more effective prophylactic treatment and an increased time of survival, noninfectious conditions associated with human immunodeficiency virus (HIV) disease are being recognized with increasing frequency in HIV patients. Cardiac involvement in HIV-infected patients varies from clinically silent to a fatal disease with a direct cardiac cause of mortality estimated at 1% to 6%. Pericardial effusion, pericarditis, myocarditis, cardiomyopathy, endocarditis, and pulmonary hypertension are known cardiac manifestations associated with HIV infection. Coronary artery disease (CAD) has not been a recognized complication of HIV disease, although some recent case reports have suggested occurrence of premature CAD and accelerated atherogenesis in HIV-infected patients. The role of protease inhibitors have been suggested in the development of this complication. After reviewing records of the last 7 years, the authors found 10 cases of acute coronary syndrome in HIV-infected patients who had no other risk factor for CAD except smoking. The presence of CAD was confirmed by angiography or autopsy. The mean CD4 count was 380 cells/mm3, and the mean duration between the diagnosis of HIV infection and CAD was 7.5 years. Four patients had single-vessel disease, 1 patient had 2-vessel disease, and 5 patients had 3-vessel disease. Three patients underwent coronary bypass surgery and 1 patient died of cardiogenic shock. CAD may be associated with HIV disease.