Spinal epidermoid cysts are rare constituting between 0.5% to 1% of all spinal tumors but accounting for up to 10% of intraspinal tumors in children. They are most often seen in the lumbosacral spine. Males are more frequently affected than females. Epidermoid cysts arise from pathologic displacement of epidermal cells into the spinal canal. They can be acquired from trauma, lumbar puncture, surgery such as myelomeningocele closure or arise as congenital lesions, like when there is improper closure of the neural tube, which results in the inclusion of epiblasts in the neural tube. Forty percent are intramedullary and sixty percent are extramedullary. Myelomeningocele is an entity which is common in the lumbosacral region due to congenital neurulation defect. The prevalence of myelomeningocele is approximately 0.8 to 1 per 1000 live births worldwide. The anatomical location may vary as cases of cervical, cervicothoracic, thoracic, thoracolumbar, lumbar and sacral have also been documented. The sac usually contains nerve bres or neural tissues, however sometimes it may contain intradural lipoma or dermoids. Here we present a rare case of a 1 year 6 month old male child presenting with with dorsolumbar myelomeningocele with symptoms of urinary and bowel incontinence and weakness of both lower limbs found to have extrameduallary epidermoid as a content of myelomeningocoele sac. The concurrence of a congenintal neoplasm within the spinal canal associated with myelomeningocele seems to be a very rare event as very few such cases have been reported worldwide to the best of our knowledge
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