AbstractWe describe a 2‐year‐old black female who was previously in complete remission from a stage 4 mediastinal germ cell tumor (stage 4 because of a single pulmonary parenchymal lesion). She presented with headaches, obtundation, and brain herniation, and suffered sudden death 12 months after completing high‐dose chemotherapy. Autopsy revealed a large intraventricular lesion with mixed germ cell tumor elements, but no other evidence of malignancy was found. This case represents the first report of isolated CNS metastasis of malignant germ cell tumor in a child in apparent remission without evidence of advanced or recurrent disease in other sites. It is possible that the aggressive chemotherapy used resulted in a change in the natural history of this disorder. © 1995 Wiley‐L