Mucormycosis is an opportunistic fungal infection mainly occurring in patients with poorly controlled diabetes mellitus or neutropenia, in recipients of corticosteroids or other immunosuppressive medications, and in those with iron overload. The infection begins in the nose and paranasal sinuses and then rapidly spreads to pulmonary, orbital, and intracranial structures. Herein, we report a case of pulmonary and rhino-orbito-cerebral mucormycosis in a patient with diabetic ketoacidosis to emphasize the importance of an early diagnosis of this potentially fatal fungal infection. We also review the recent literature on the management of mucormycosis.