Since Askanazy's first report on a case of primary intracranial choriocarcinoma in 1906, more than 60 cases have been reported so far. We add to that a case of intrasellar choriocarcinoma with suprasellar extension: A 12-year-old girl showed cranial nerve palsies, hypopituitarism, and abnormally high titers of human chorionic gonadotropin (HCG) in blood and in cerebrospinal fluid (CSF). She died 3 months after surgery. At autopsy, an intra- and suprasellar choriocarcinoma, which penetrated through the clivus into the cerebellopontine angle and metastasized remotely to the lungs, was confirmed. This rare case is presented with a thorough review of the literature.