We report a case of combined microcystic adenoma and pancreatic endocrine neoplasm of the pancreas in a 53-year-old male patient. The pancreatic tumor was an incidental computed tomography scan finding and was not accompanied by gastrointestinal symptoms. The tumor was located in the head of the pancreas and was composed of numerous small cysts lined by uniform clear cells with a centrally located solid endocrine component. Four cases of similar neoplasm have recently been reported, exclusively in women. Literature review and case analysis indicate that combined microcystic adenoma and pancreatic endocrine neoplasm is characterized by the presence of pancreatic endocrine neoplasm within microcystic adenoma in the head of the pancreas, affects women more often than men, and presents at a younger age when compared to microcystic adenoma.