A case of malignant solitary fibrous tumor (SFT) is reported, occurring in a 61-year-old man with frequent hypoglycemia. Endocrinological analyses showed high serum levels of insulin-like growth factor II (IGF-II) and suppressed secretion of insulin. After the removal of a pleural tumor, which weighed 3150 g, serum IGF-II levels returned to normal and hypoglycemic attacks ceased. The tumor was composed of uniform spindle cells arranged in bundles, and fascicles with varying amounts of collagen and reticulin fibers. Mitotic figures at the rate of 6/10 high-power fields, and frequent foci of necrosis and hemorrhage were seen. Almost all of the tumor cells were immunohistochemically positive for vimentin and CD34. Electron microscopy revealed the immature mesenchymal or myofibroblastic nature of the tumor cells. These findings are consistent with malignant SFT of the pleura. Moreover, the tumor produced IGF-II mRNA as demonstrated by northern blot analysis. Thus, hypoglycemia of this patient was induced by SFT through the production and secretion of IGF-II.