Epidermoid cysts are rare, benign neoplasms that account for less than 1% of all intraspinal tumors. The most common localization is in the lumbar area, and one-third of the tumors are intramedullary. In this video, the authors present removal of a thoracic intramedullary epidermoid tumor in a 6-year-old boy, carrier of a 22q11 gene duplication and affected by psychomotor retardation. He presented a 1-year history of progressive gait impairment. No history of lumbar puncture or trauma was reported. The procedure was performed under neurophysiological monitoring, and it was uneventful with complete recovery of neurological function. Technical nuances are illustrated.