We report a case of pancreatic endocrine tumor admixed with a diffuse microcystic adenoma in a 67-year-old woman with a six months history of melena. Whipple's operation and near-total pancreatectomy were performed under the impression of a pancreatic cancer with duodenal invasion. The pancreas was enlarged and was entirely replaced by sponge-like tiny cysts, which were lined by a single layer of periodic acid Schiff positive cuboidal cells. Also encountered was a gray white solid area at the head portion which extended to the duodenum with an intraluminal polyp formation. The compact area was composed of solid and acinar structures of tumor cells, which showed a positive reaction for chromogranin A and neuron specific enolase, separated by delicate highly vascularized stroma. To the best of our knowledge, this is the first case report published with immunohistochemical studies to deal with a pancreatic endocrine tumor admixed with a diffuse microcystic adenoma.