STUDY DESIGN A case report and review of the literature are presented. OBJECTIVES To describe the clinical course and treatment of a patient with an unusual intraosseous degenerative cyst within the body of the axis, as well as review the literature regarding these lesions. SUMMARY OF BACKGROUND DATA Intraosseous degenerative cysts of the cervical spine are extremely rare. To our knowledge, only 4 prior case studies have described these lesions, 2 of which were seen in the body of C2. METHODS A case report of a 58-year-old patient with neck pain and an intraosseous cyst within the axis is presented with a review of the pertinent literature. RESULTS History, examination, radiographic evaluation, and histology revealed this lesion to be an intraosseous degenerative cyst within the body of C2. The patient was treated with anterior surgical biopsy/curettage and posterior stabilization with structural graft enhancement. CONCLUSION Although rare, intraosseous cervical degenerative cysts should be present in any differential diagnosis of cystic lesions seen in the cervical spine.