Craniopharyngioma is a benign intracranial tumor that can be clinically aggressive. The descriptive epidemiology of this tumor is not fully known, in part because of its borderline nature between a benign and malignant tumor, and in part because of its rarity. It has a bimodal age distribution with a peak between 5 and 14 years of age, and a second one in adults older than 65 years. The Childhood Cancer Registry of Piedmont, Italy, estimates an incidence of 1.4 cases per million children per year. Similar data are provided by other registries in Western countries, while higher rates have been observed in Asia and Africa. There are no known specific environmental risk factors for craniopharyngioma, and genetic predisposition is not demonstrated. Survival has dramatically improved in the last years.