Intradural extramedullary (IDEM) ependymoma except for tumors originated from the filum terminale or conus medullaris is rare. The present study showed a case of IDEM ependymoma. A 16-year-old boy was referred to our hospital with a complaint of right hypochondriac pain and motor weakness in his right leg. MRI revealed a solitary intradural tumor at Th5-8 level with syringomyelia at Th2-4 level. Microscopic total tumor resection was performed with right hemi-laminectomy of Th4-9. Histological diagnosis was ependymoma (WHO grade 2). Although his leg weakness was worsened transiently, he showed improvement in leg weakness being able to go up and down the stairs 1 month after the surgery. There was no tumor recurrence until now, 7 years after the surgery, without any adjunctive therapies. A total of 44 cases of IDEM ependymoma had been reported in the past literatures. They are thought to arise from ependymal cells which remained during the process of neural tube closure. Like intramedullary ependymomas, most of the IDEM ependymomas have clear border to surrounding tissue and often removed completely. However, a small number of recurrences and malignant transformations had been reported after complete resections despite benign histological features tumors. In the case of totally resected low grade IDEM ependymoma, it is thought to be reasonable to perform long-term periodical radiographic follow-up without postoperative adjunctive therapy.