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Congenital generalised Berardinelli-seip lipodystrophy is a rare, autosomal recessive disorder characterised by selective absence of adipose tissue. Affected individuals are predisposed to severe insulin resistance and its attendant complications, including diabetes mellitus, hypertriglyceridaemia, acute pancreatitis and hepatic steatosis. The management of diabetes in these people can be challenging due to severe insulin resistance.A 25-year-old female patient, with diabetes secondary to congenital generalised lipodystrophy, presented to the emergency department with a two-week history of sudden onset, spontaneous left leg pain associated with swelling over the medial aspect of her left thigh. Her diabetes control prior to presentation was unsatisfactory with HbA1c >12%, despite receiving maximum doses of metformin, pioglitazone and 200 units of subcutaneous insulin daily.Based on the clinical presentation, background medical history, examination and MRI findings, a diagnosis of diabetic muscle infarction was made. The patient's symptoms resolved over the next four to six weeks with rest and analgesia. Eleven months later, she represented with diabetic muscle infarction affecting her left quadriceps and, after a further 19 months, she had a third admission to hospital with diabetic muscle infarction but this time affecting her right quadriceps.We describe a rare case of recurrent diabetic muscle infarction. This complication has been previously reported in patients with type 1 diabetes of prolonged duration; however, we are not aware of any report of diabetic muscle infarction occurring in patients with diabetes secondary to congenital generalised lipodystrophy. In the current report, we discuss the pathogenesis, clinical course and management of diabetic muscle infarction.