A 57 year old male presented with a ten-year history of diabetes, a three-year history of recurrent peptic ulcer, and a two-year history of painful glossitis and skin rash. A CT scan disclosed a tumor of the pancreatic tail. Preoperative studies revealed elevated levels of glucagon, gastrin, pancreatic polypeptide, and pancreastatin. A distal pancreatectomy with en bloc splenectomy was performed and resulted in resolution of symptoms.