&NA;A case of embryonal carcinoma in the pineal region of a 17‐year‐old boy is presented. The tumor included elements of choriocarcinoma and endodermal sinus tumor, and the use of human chorionic gonadotropin and alpha‐fetoprotein as tumor markers is discussed. The markers were demonstrated both within the tumor and in the cerebrospinal fluid (CSF) and blood. The patient was treated with a postoperative program of irradiation and cancer chemotherapy, and at follow‐up examination 20 months after operation no signs of residual tumor were present. It is suggested that human chorionic gonadotropin and alpha‐fetoprotein should be measured in the blood and CSF before the treatment of midline tumors.