OBJECTIVE: To systematically review all the reported cases of diabetic muscle infarction (DMI) and its pathogenesis, clinical features, prognostic implications, and management. RESEARCH DESIGN AND METHODS: We searched databases (MEDLINE and EMBASE) from their inception to August 2001 and reviewed bibliographies in reports retrieved. Data were extracted in a standardized form. RESULTS: A total of 47 references were retrieved; 115 patients and 166 episodes were included. DMI was more frequent in women (61.5%, mean age at presentation 42.6 years). Of the cases, 59% had type 1 diabetes; the mean duration of disease was 14.3 years, and multiple diabetic end-organ complications were noted. DMI affects the lower limbs with abrupt onset of pain and local swelling. Diagnosis is made by biopsy, but the characteristic features in magnetic resonance imaging are very typical. Treatment includes bed rest and administration of analgesics, but recurrence is common. CONCLUSIONS: DMI is a very uncommon complication of long-standing diabetes; presentation is well characterized and management is simple.