A large, highly ossified craniopharyngioma was diagnosed and surgically treated in a 2-year-old girl. About 20 teeth were macroscopically identified in the operative specimen, some of them totally developed, complete with crown and root. Pathological study established the diagnosis of craniopharyngioma with formation of teeth. Only five cases have been reported previously, and only one patient survived the operation. The patient is alive 3 years after surgical treatment. Radiotherapy was not used.