Objective: Primary spinal cord tumors are very rare (incidence 0.26/100,000 person/years) in children and adolescents (0-19 years), commonly presents with low back pain, pain radiating to thigh/knee, lower extremity numbness/weakness, urinary dysfunction and abnormal gait. Here we present a case of spinal cord ependymoma in sacral region presented with neurogenic bowel which is very rare in paediatric age group. Methodology: A 5 years old girl from Dhaka presented with increased bowel frequency, 8-12 times/day, semi solid for 2 years without any systemic or neurological complaints. On query, her parents disclosed history of urinary retention 2 years back which improved with intermittent self-catheterization over 3 months with subsequent normal bladder habit for 2 years. General examination, vital parameters were normal. Anal area appeared normal with reduced anal tone. Neurological examination including motor, sensory examination of both lower limbs and other systems revealed normal. Results: CBC, RBS, TSH, creatinine, electrolytes, CXR, USG of whole abdomen, stool R/E, culture, colonoscopy were normal. MRI of lumbo sacral spine (sagittal precontrast T1W1)(Figure1a) revealed a heterogenous, iso-intermediate intense (in comparison to spinal cord) lobulated mass at S2-S5, with mild inhomogenous enhancement on post contrast T1W1(figure1b). It was clearly delineated as mild heterogenous, hyperintense lobulated lesion in Sagittal T2W1(figure1c) suggesting intracanalicular myxopapillary ependymoma/ Schwannoma/ neurofibroma involving S2-S5 spine. Conus medullaris(L1), disk heights were normal without any sign of tethered cord or disc herniation. After laminectomy an extradural tumour (S1-S4) was removed near totally(Figure1de), histopathology revealed cellular ependymoma (WHO grade II). Leukocyte common antigen, CD20, CD3 was negative. It was followed by palliative Radiotherapy (total T.D 4400cGy,22Fr,31days). Conclusion: Patient regained her normal bowel habit within 6 months of surgery. After 8 years she was completely symptoms free without any tumor recurrence which supports long term survival and complete functional recovery of spinal cord ependymoma with specific therapy.