We describe a 29-year-old woman who presented with progressive neck pain, sensory deficit and weakness in both arms. Magnetic resonance imaging (MRI) of the cervical spine revealed an extramedullary tumor with severe spinal cord compression. During surgery an intradural extramedullary tumor was found. Further imaging showed a second lumbar spinal tumor. Microscopy of both tumors showed that both tumors were anaplastic ependymomas, which almost never present as extramedullary tumors. Two years after surgery, an intracranial extracerebral metastasis was found, without evidence of spinal recurrence.