A 42-year-old woman was hospitalized for severe hypoglycemic coma. She had a voluminous hepatic metastasis and multiple small lung metastases from a meningeal hemangiopericytoma initially operated on 11 years earlier. High blood levels of an abnormal form of insulin-like growth factor type 2 (IGF II) associated with low blood levels of insulin, growth hormone, IGF I, and IGF BP3 were observed. After surgical resection of the liver and pulmonary metastases, serum glucose levels and hormonal abnormalities returned to normal.