Two cases of endodermal sinus tumor of the pineal region are reported and compared with 16 others in the literature. The preponderance of neoplasms occurs in boys, usually in their second decade. The diagnosis is usually established within three months of the onset of non-specific symptoms of an intracranial mass. Treatment with operation or radiotherapy alone is inadequate. Most patients have died within two years, some with widespread intradural metastases. The one individual who has survived five years was treated with both operation and radiotherapy.