A Case report about an intramedullary epidermoid cyst and its association with an arrested ascent of the spinal cord, concomitant the neurological deficits, the surgical treatment and the clinical progress after one year. Epidermoid cysts are rare in the spinal cord, less than 1% of all spinal tumors. A 40-year-old woman described hypesthesia in the right left foot and back pain since one year. The MRI provided the evidence of an intramedullary epidermoid cyst at the level of L1/2 and revealed an arrested ascent of the cord opposite vertebra L3. With total compression of the spinal cord so we decided to operate on her. Intraoperative the intramedullary tumor proved to be an epidermoid cyst with typical pearly appearance and the presence of hair. The surgical removal including capsule was uncomplicated done under neurophysiological monitoring. The hypoesthesia was obviously regressive in the following postoperative days, as well as severe back pain. The MRI after 12 months revealed a fractional cystic alteration. The patient maintained no complaints up to lightly hypesthesia in the right foot. The total resection of epidermoid cyst to avoid recurrence is the main treatment. Neurophysiological monitoring is mandatory.