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Seth G.S. Medical College @ K.E.M. Hospital, Department of Neurosurgery, Mumbai, India DOI : 10.5137/1019-5149.JTN.3096-10.2 Epidermoid cysts are benign tumors originating from ectoderm remnants. Most epidermoid cyst cases are intracranial. Spinal epidermoid cysts are uncommon and most of the reported cases are in the thoracic and lumbar spine. Occurrence of intramedullary epidermoid cysts in the cervical spine is extremely rare. An 18-year-old male with a giant intramedullary tumor extending from the brain stem to the upper thoracic spinal cord presented at our outpatient department. The patient underwent total excision of a silvery white lesion through a midline myelotomy. Histopathological examination was suggestive of an epidermoid cyst. We present a brief report of the case and discuss the relevant literature. Keywords : Epidermoid, Intramedullary, Spinal cord