We report a 24-year-old woman with an intramedullary conus ependymoma associated with holocord syringohydromyelia and syringobulbia. The tumor was removed and surgery for decompression of the syringohydromyelia was not considered at the first operation. In the follow-up examinations, MRI showed significant and steady improvement of syringohydromyelia. Symptoms associated with syringohydromyelia also disappeared. The cause of syringohydromyelia accompanying intradural spinal cord tumors appears to be either direct blockade of the central canal or secondary interruption of the central canal flow by compression of the perimedullary cerebrospinal fluid flow. As removal of the mass often corrects both these likely causes of the syringohydromyelia, no additional treatment for the drainage of the hydromyelia cavity syrinx is usually necessary. (c) 2006 Elsevier Ltd. All rights reserved.