We report a case of rhinocerebral mucormycosis presenting in a patient with AIDS and review the literature on mucormycosis occurring in the setting of HIV disease. Mucormycosis in HIV is rare. However, it can be the presenting opportunistic infection in AIDS. Predisposing factors for Mucor infection in HIV disease include low CD4 count, neutropenia, and active intravenous drug use. Mucormycosis can present in the basal ganglia, the skin, the gastrointestinal tract, the respiratory tract, or may be disseminated. The disease may develop insidiously or may progress rapidly with a fulminant course. Therapy usually consists of surgical debridement/excision accompanied by intravenous amphotericin B.