Introduction: Spinal epidermoid accounts for < 1% of all primary cord tumor. This rare benign lesion may be congenital or acquired. Invagination of epidermal elements into the neural tube during embryonic period leads to the development of epidermoid cyst. Case Presentation: A 2 year old girl presented with a spontaneous intergluteal swelling with dermal sinus and lower limbs weakness. The lumber MR demonstrated an intradural lesion from L3 to L5 levels that compressed cauda equine or nerve roots. MR findings were compatible with an epidermoid cyst with dermal sinus without any coexistent spinal dysraphism. The patient underwent microsurgical laminectomy from L3 to L5, tumor was resected and sent for histopathological examination. A pearly white tumor was found and histopathology revealed an epidermoid tumor. Post-operative Magnetic Resonance Imaging (MRI) finding was total removal. Conclusion: Diagnosis of spinal epidermoid cyst is often delayed due to its obscure presentation. Here, we presented spinal lumber intradural epidermoid cyst with dermal sinus which was treated successfully with microsurgical total resection with preservation of spinal stability and neurological function. Post-operative follow up with MRI is helpful.