Mucormycosis is a highly aggressive fungal infection affecting diabetic, immunocompromised, and, occasionally, healthy patients. This infection is associated with significant mortality. We have reviewed 208 cases in the literature since 1970, 139 of which were presented in sufficient detail to assess prognostic factors, and added data from six of our patients. The histories of these 145 patients were analyzed for the following variables: 1) underlying conditions associated with mucormycotic infections; 2) incidence of ocular and orbital signs and symptoms; 3) incidence of nonocular signs and symptoms; 4) interval from symptom onset to treatment; and 5) the pattern of sinus involvement seen on imaging studies and noted at the time of surgery. Factors related to a lower survival rate include: 1) delayed diagnosis and treatment; 2) hemiparesis or hemiplegia; 3) bilateral sinus involvement; 4) leukemia; 5) renal disease; and 6) treatment with deferoxamine. The association of facial necrosis with a poor prognosis fell just short of statistical significance, but appears clinically important. This is the first review that documents the heretofore intuitive claim that early diagnosis is necessary to cure this disease. Standard treatment with amphotericin B and aggressive surgery are reviewed and adjunctive therapeutic modalities are discussed, including local amphotericin B irrigation, hyperbaric oxygen, and optimizing the immunosuppressive regimen in transplant patients. Hyperbaric oxygen was found to have a favorable effect on prognosis. In addition, possible treatment options for patients with declining renal function are reviewed.