Background Data:Myxopapillary ependymoma (MPE) is a low-grade variant of ependymoma that typically occurs in conus medullaris and filum terminale of the spinal cord. Only rarely do these tumors present at an extradural location in the sacrococcygeal region and even rarer is the development of these extradural tumors inside the spinal canal. The authors report a case of an intraspinal extradural MPE of the sacrum with relevant literature review on salient features and management. Study Design: Case Report. Purpose: To report a rare entity at an uncommon location. Case Report: A 12-year-old male child presented with chief complaints of low backache and radiation of pain in both lower limbs for six months. The patient also had a history of straining during micturition for one month. On examination, he was found to have mild weakness of bilateral ankle plantar flexion and knee flexion (grade 3). Ankle jerks were absent and there was evidence of sacral hypoaesthesia. Intraoperatively sacral lamina was found to be thinned out. Laminectomy was performed at L5 and sacrum, an extradural, fleshy lesion extending from L5 to S5 was identified, and gross total resection was achieved. There was no intradural expansion of the tumor. Results: Patient had an uneventful course and was discharged home on day 4. Conclusion: Intraspinal extradural MPE is a rare entity and should be kept in mind while diagnosing a midline lower back lesion. Treatment consists of gross total excision wherever possible coupled with radiotherapy. These tumors tend to recur locally and systemically and, thus, patients should be followed up periodically. (2020ESJ226)