To investigate the role of surgical tumor resection, radiotherapy and chemotherapy, the outcome of treatment in 17 children with nongerminomatous malignant germ cell tumor (NG-MGCT) was reviewed. The median follow-up period was 38 months after diagnosis, and the overall 3-year survival rate was 75%. Eleven patients who underwent craniospinal radiation (CSRT) did not receive chemotherapy. In 4 of them more than 90% of the tumor was removed, and they were free of disease at 16, 30, 93 and 111 months after surgery. Among the other 7, who did not undergo tumor resection (n=5) or had considerable residual tumor (n=2), 2 were disease-free at 73 and 88 months after diagnosis, and 5 died of recurrences. Of 6 patients who received cisplatin and etoposide chemotherapy in addition to CSRT, none showed intracranial recurrence, regardless of the extent of removal. The authors believe that multimodal treatment is the preferred choice and that chemotherapy plays an important role, especially when a significant amount of tumor remains after surgery. CSRT plays a major role at least in some patients. If chemotherapy is not feasible, radical removal plus CSRT seems to be an alternative.