We describe three cases of cerebral mucormycosis in intravenous drug users and review 22 previously reported cases. Involvement of the basal ganglia was demonstrated in all but two cases. Seven of the 10 patients tested for antibodies to the human immunodeficiency virus (HIV) were seronegative. Eight of the 25 patients survived and were discharged from the hospital; for 7 of 10 patients, cultures of brain lesions yielded Rhizopus arrhizus. The radiographic findings varied, and in most cases, no or minimal contrast enhancement was seen in the initial computed tomography scans. Although uncommon, the diagnosis of cerebral mucormycosis should be considered when basal ganglia lesions are present in an intravenous drug user, regardless of previous exposure to HIV.