This case report concerns an endodermal sinus tumor (EST) arising in the pineal region of a 16-year-old boy who died 3 months after radiation. He developed extensive abdominal metastases through a ventriculoperitoneal shunt, whereas the primary tumor and a suprasellar metastasis could be controlled by radiotherapy, demonstrated by autopsy. The histologic diagnosis was supported by an elevated level of alpha-fetoprotein in serum and the demonstration of this marker in the tumor tissue by immunoperoxidase method. The poor diagnosis of all previously reported cases with pineal EST requires a combined modality of surgical approach, radiotherapy, and concomitant chemotherapy.