Maffucci's syndrome is a rare congenital mesodermal dysplasia combined with dyschondroplasia and hemangiomatosis. About 150 cases have been reported till now. Maffucci's syndrome is often combined with other neoplasms. Herein, we report a patient with Maffucci's syndrome and hemangiomas on the dorsum of the tongue, which is rare in this syndrome.