The upper cervical region and in particular the atlas remain very rare locations for osteoid osteomas. Herein a 30-year-old male with osteoid osteoma of the atlas arch and a history of severe neck pain for a year is presented. After establishment of the diagnosis by isotope and C.T. scan, tumor removal was accomplished through atlas arch laminectomy resulting in complete relief of pain. A literature review revealed that only four similar cases have been reported previously.