Introduction: Mucormycosis is a rare, fatal infection which mainly affects the patients with diabetes or immunodeficiency. Various clinical forms of the disease include rhinocerebral, pulmonary, cutaneous, gastrointestinal, and disseminated infection. The aim of this study was to report a rare case of mucormycosis involving middle ear.

Case Report: A 22-year-old female patient with a history of type 1 diabetes mellitus and end stage of renal disease who were under treatment with insulin and twice a week hemodialysis refered to hospital because of abrupt onset of severe left ear pain, and received oral antibiot-ics. Her symptoms worsened and left facial palsy developed the day after. Myringotomy was done and administration of parenteral antibiotics were begun. Subsequently, she developed left fifth and sixth cranial nerve palsies, followed by fever, unconsciousness, left hemi-facial edema, and serosanguinous discharge from eye and nose. In spite of parenteral antibacterial and antifungal therapy, she developed refractory septic shock and died.

Conclusion: Acute otitis media in diabetic patients may be due to mucormycosis. Considering the rapid progression of necrosis and fatality, prompt diagnosis and therapy for mucormycosis is mandatory.