Glucagonomas are rare tumors. They are predominantly located in the body or tail of the pancreas and display a constellation of signs and symptoms referred to as glucagonoma syndrome. The term necrolytic migratory erythema is used to characterize the distinctive rash associated with this syndrome. This report describes a classic presentation consisting of dermatitis, glossitis, stomatitis, angular cheilitis, anemia, and weight loss that was associated with the finding of a pancreatic mass and a markedly elevated plasma glucagon level. After pancreatic resection, the patient had complete resolution of the rash and normalization of plasma glucagon.