The literature on zygomycotic infection in HIV-positive patients is reviewed. A total of 28 patients have been reported. The risk factor for HIV infection was known in 22 of these and was sexual transmission in only six patients and intravenous (i.v.) drug use in 16 (73%). In the latter group, it is likely that i.v. drug injection was the entry route of the zygomycotic infection. The most prevalent clinical presentations of zygomycosis in the i.v. drug-using HIV-positive patients were cerebral, cutaneoarticular and renal (accounting for 88% of presentations in total). These presentations were much less common (18%) in a cohort of 116 patients belonging to the conventional risk groups for zygomycosis (HIV negative and no i.v. drug use). The isolated cerebral localization is the typical presentation of zygomycosis in HIV-negative i.v. drug users.