The occurrence of malignant lymphoma in the pineal region is very rare. We experienced a case of pineal region tumor that was treated by radiation and chemotherapy. After 1 year, the tumor metastasized to the cauda equina and was operated on. The diagnosis was malignant lymphoma. Although the incidence of intracranial malignant lymphoma is known to be on the increase recently, malignant lymphoma should be considered in the differential diagnosis of pineal region tumors.