Glucagonoma is a very rare islet cell tumor of the pancreas. We present a case of pancreatic tail tumor with the typical glucagonoma syndrome of necrolytic migratory erythema (NME), diabetes mellitus (DM), anemia, weight loss and glossitis. After complete resection of the pancreatic tumor, the glucagonoma syndrome subsided. In reviewing 120 cases of glucagonoma in the literature, the average tumor diameter was 3.6 cm. Most (68.1%) of the tumors occurred in the pancreatic tail. Two-thirds of the reported glucagonomas were malignant and 53.5% metastasized to other organs. The curative resection rate was 45.8%. A triad of pancreatic tumor, NME and DM should lead to the diagnosis of glucagonoma.