Ewing sarcoma is a rare neoplasm that mostly grows in bones or soft tissues around bones. Most cases of Ewing sarcoma occur in intra-skeletal locations (long bones, pelvis, or ribs) and rarely in extra-skeletal regions such as paravertebral or epidural space, whereas a primary intradural extramedullary Ewing sarcoma (IEES) is extremely rare. Fifty cases of primary IEES including our case were identified in the literature, so far, of which two-thirds are in the lumbosacral region, while only 12 reports described a cervical location of the tumor. Herein, we are presenting a case of C7-T1 primary IEES for a 24-year-old male, followed by a review of updated literature about the primary IEES in the cervical spine.