A primary intracranial ependymoblastoma presented only with symptoms of spinal cord compression due to metastatic spread of the tumor via the leptomeninges. Such a presentation is very rare among primary neoplasms of the central nervous system (CNS) and unique among the 12 previously reported ependymoblastomas. The previous cases all arose as primary brain tumors and several were remarkable for extensive leptomeningeal spread of the tumor. These observations and the presentation of our patient suggest that a histological diagnosis of ependymoblastoma of the spinal cord, with or without symptoms of intracranial tumor, should suggest that an intracranial primary lesion might be present.