Epidermoid cysts are composed of stratified squamous epithelium, which develop from inclusion of ectodermal tissue remnants entrapped during primitive neural tube closure period between the 3rd and 5th weeks of gestation. Intramedullary epidermoid cysts (IEC) are very rare in childhood. Eleven cases have been reported previously. Even though it is a rare entity, early diagnosis with timely fashioned surgical treatment has a great influence on patients' symptoms, especially if there are myelopathic findings. In this case report, we demonstrate this fact with a demonstrative clinical case and review radiological, pathological, and surgical perspectives with literature review.