The normal islets of Langerhans (pancreas) contain four major cell types, 15-20% of which are alpha cells which produce glucagon.1 Alpha cell tumors can be divided into two distinct types; those associated with glucagonoma syndrome2 (necrolytic migratory erythema, diabetes, anemia, stomatitis, weight loss, venous thromboses, diarrhea, and sometimes psychiatric disturbances), and those which are not. Those associated with the syndrome tend to be large and solitary and there is a high incidence of malignancy. Those glucagonomas not associated with the syndrome tend to be multiple and small and are nearly always benign.3 Immunohistochemically, the two types of glucagonoma are reactive to the glucagon antibody, though occasionally only weakly.