Diabetic amyotrophy is a rare complication of diabetes compared to distal symmetric polyneuropathy, but can occasionally be encountered in clinical practice, particularly as the incidence of diabetes increases. The distinctive history of unilateral neuropathic symptoms followed rapidly by atrophy and weakness is typical of the disorder. This complication most commonly occurs in cases of well-controlled Type 2 diabetes mellitus. While the underlying pathophysiology is known to be microvasculitic in nature, the diagnosis is often based on clinical and electrodiagnostic grounds and tissue biopsy is not typically performed. Attempts at corticosteroid administration during immunotherapy should be carefully considered on a patient-by-patient basis. Better recognition of this disorder is likely to result in more rapid diagnosis, counselling, and subspecialty referral.