Mucormycosis is an aggressive and frequently lethal disease. Most patients with mucormycosis have poorly controlled diabetes mellitus and rhino-orbito-cerebral disease. Patients with hematologic malignancy and transplant recipients mostly present with rhino-orbito-cerebral or pulmonary disease. Prompt recognition of clinical symptoms and radiographic features of mucormycosis is required to establish timely diagnosis and initiate targeted therapy. Diagnosis is, historically, made by direct microscopy, culture, and pathology of biopsy tissue, but molecular methods are increasingly playing a role in establishing an earlier diagnosis. Treatment is multidisciplinary, involving early surgical intervention, antifungal therapy, and correction of underlying immune compromising risk factors when possible.