A 31-year-old male developed intramedullary tumors in the medulla oblongata and the upper cervical spinal cord. He was first admitted with tetraparesis. Magnetic resonance (MR) imaging revealed a low intensity mass lesion in the medulla oblongata. The tumor was removed and diagnosed as a pilocytic astrocytoma. Nine years later, he was readmitted with motor weakness and dysesthesia in the right arm. MR imaging revealed a mass lesion in the cervical cord. This tumor was removed and diagnosed histologically as ependymoma. We suggest that the displacement of primitive spongioblasts with subsequent differentiation resulted in an astrocytoma and an ependymoma in adjacent areas.