A 26-year-old man of Polish origin, previously fit and healthy, presented with a ten-day history of pain in the left leg and lower back. This was followed by difficulty in urinating and decrease in sensation in both legs. Examination revealed pyramidal signs and mild weakness of both lower limbs. He had early sphincter involvement requiring catheterization. Magnetic resonance imaging (MRI) of the brain was normal. However, that of the spinal cord revealed multiple intradural spinal lesions, both intra- and extramedullary, extending from the cervical cord down to the cauda equina roots. There was also a syrinx extending from T1 to T10. There were no intracranial lesions. A T12-L1 laminectomy was performed. Multiple intradural, extra- and intramedullary tumors were seen. An exophytic intramedullary tumor arising from the conus was biopsied which was confirmed to be an ependymoma. Histology showed a moderately cellular tumor with typical perivascular rosettes. Ki-67 showed a low and/or moderate cell turnover, suggestive of primary typical ependymoma. There was no evidence of anaplastic change. After the operation, the patient deteriorated with a sensory level at T4. Post-operative cranio-spinal radiotherapy was administered, but there was no clinical improvement in the lower limbs.