A case of malignant ependymoma with extracranial metastases is reported. A 59-year-old male was admitted to our hospital with vomiting and ataxia. Following computed tomographic (CT) scanning indicating a ring-like enhanced mass in the cerebellum, the tumor was subtotally removed in December, 1985. Histological diagnosis was malignant ependymoma. A second operation was performed in February, 1987, due to recurrence of the tumor in the fourth ventricle. Postoperative radiation therapy at a total dose of 7780 rads was given over 2 years. Eleven months after radiation therapy was completed, he reported cough and multiple skin metastases. Chest x-rays showed metastases at the right hilus. Repeated CT scans revealed separate frontal cerebral metastatic tumors. He died of respiratory insufficiency. Extracranial metastases of infratentorial ependymoma to the skin and lung are rare pathological entities.