A report is given on a 14-year-old boy with an ependymoma of the brain which was 3 times operated upon. At autopsy, a widespread leptomeningeal and intraventricular dissemination was found. Extraneural metastases developed in the soft tissues of the neck as well as in the pleura and lung on the left side. Immunohistochemically, the cells of the brain tumor and extraneural metastases of the neck showed a positive GFAP-reaction. According to Tables 17 to 19 of Jänisch and co-workers (1976; 1988) and Table 1 of this paper, 48 intracranial, intraspinal and ectopic (subcutaneous sacrococcygeal) ependymomas with extraneural metastases were collected from the literature.