Atlantoaxial facet joint osteoarthritis is rare, often undiagnosed because it may be misdiagnosed as occipital neuralgia, or degenerative cervical spondylosis. Unilateral occipitocervical pain aggravated by head rotation is a specific symptom. Conservative treatment is usually effective. But when the patient complains of intractable neck pain localized to occipitocervical junction and unresponsive to medical therapy, surgical treatment should be considered. Though a few reports of surgically treated atlantoaxial osteoarthritis has been published, surgical outcome is favorable. A case of a surgically treated atlantoaxial osteoarthritis is presented with a review of the literatures.