In 1903 Erdheim reported multiple endocrine neoplasia type 1 (MEN 1) by autopsy, and in 1954 Wermer reported a familial occurrence of multiple tumors which were associated with neoplastic transformation of parathyroid, pituitary and pancreatic islet cells. This complex association of abnormalities is inherited as an autosomal dominant trait and related to tumor suppressor gene on chromosome 11. In a 38-year-old woman with epigastric discomfort, general weakness and mental change, a pituitary gland tumor was diagnosed with sella magnetic resonance imaging and combined pituitary stimulation test. a pancreas insulinoma, an adrenal gland cortical adenoma and a thyroid adenoma were confirmed by pathology and they were diagnosed with biochemical test, CT scan, percutaneous transhepatic portal vein catheterization with insulin sampling, thyroid sonogram and scintigram. The blood glucose level was normalized after operation. In the 6 months follow up study, she has not presented any symptoms of hypoglycemia so far. Authors present this case briefly with a review of literature.