A 55-year-old man presented with an 11-year history of necrolytic migratory erythema and glossitis. After the patient's serum glucagon was demonstrated to be elevated, computed tomography scan revealed a mass involving the head of the pancreas. The patient underwent a Whipple-type pancreatico-duodenectomy and his rash resolved completely 6 days after tumor resection. He received no adjuvant treatment. A discussion of the varying theories regarding the pathogenesis and treatment of glucagon-associated necrolytic migratory erythema is presented.