A 65-year-old man presented with a case of pure yolk sac tumor in the pineal region. The patient was admitted with a gait disturbance and elevated alpha-fetoprotein levels in serum and cerebrospinal fluid. He underwent total resection of the tumor. Cisplatin-vinblastine-bleomycin therapy was interrupted because of severe syndrome of inappropriate secretion of antidiuretic hormone, and there was recurrence of the tumor. Five months after the operation, he died in spite of carboplatin-etoposide therapy. A review of the literature revealed no previous report of pineal yolk sac tumor in an elderly patient. Problems in the diagnosis and treatment of such cases are discussed.