Isolated central nervous system (CNS) mucormycosis is a rare but life-threatening infection. We report a case of bilateral frontal lobe mucormycosis in a previously healthy woman. Intravenous drug use was the most likely route of infection. After treatment with surgical drainage and amphotericin B, she survived but suffered permanent neurologic deficits. We identified 29 previous reports of isolated CNS mucormycosis in the medical literature. Including our case, the patients averaged 32 years of age and most were male. Intravenous drug use appears to be the most important risk factor, present in 20 patients (67%). Treatment with amphotericin B was the only predictor of survival, reducing mortality from 92% to 41%. Isolated CNS mucormycosis should be considered in the differential diagnosis of brain abscesses, especially in intravenous drug users. Prompt initiation of therapy with amphotericin B may be life-saving.