Background Although neurological complications of human immunodeficiency virus (HIV) infection are common, the presence of cerebrovascular disease (CVD) has been seldom reported. The purpose of this report is to review available data on the association between stroke and acquired immunodeficiency syndrome (AIDS). Summary of Review A review of all literature published between mid-1976 and December 1994 was performed through a MEDLINE search with the following key words: AIDS, CVD, human T-cell lymphotropic virus type III, and HIV-1. Only reports of clinical stroke in patients with AIDS or HIV infection and autopsy series with stroke findings were selected. The type of study, population, number of stroke patients, subtype and etiology of stroke, and associated AIDS conditions were described. Six clinical series and 11 autopsy series were found, with a total of 1885 cases with AIDS, AIDS-related complex, and HIV carriers. Forty percent had a neurological complication, but only 1.3% had a stroke syndrome. Ischemic infarcts were more common than intracerebral hemorrhages. Cerebral infarcts were generally due to nonbacterial thrombotic endocarditis or concomitant opportunistic central nervous system infection, and intracerebral hemorrhages were usually associated with thrombocytopenia, primary central nervous system lymphoma, and metastatic Kaposi’s sarcoma. Autopsy findings of CVD were generally not related with clinical stroke before death. Data are not available to determine the role of risk factors for AIDS in CVD. Conclusions Because of limitations of the available data, it is still not clear whether there is an association between AIDS and stroke. Further studies are needed to better define the epidemiology of CVD in association with AIDS.