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Ewing’s sarcoma is a rare cause of spinal compressive, myelopathy. Its presence in the craniovertebral junction is even rarer. The presence of both with reversable myelopathy after surgery makes this a truly rare and fortunate instance of prompt neurosurgical decompression coupled with judicious use of adjuvant therapy to affect the best possible results for the patient. We present our experience in this case mentioned later along with a 10-year review of literature regarding the incidence, distribution and presentation of cervical spinal Ewing’s sarcoma to further demonstrate the rare nature of this remarkable case report.