Abstract： Objective To present a clinical and radiological evaluation of 22 consecutive patients with two kinds of cranio-cervical junction malformation who were managed in a one-stage operation with different approaches and methods. Methods Admitted into the Third Affiliated Hospital of Peking University from January 2002 to July 2007, 22 consecutive patients with two kinds of cranio-cervical junction disorders (congenital atlanto-occipital malformation accompanied by acquired cervical spondylotic myelopathy) underwent clinical, radiological and MRI evaluations. Clinical assessment consisted of a detailed history and neurological assessment and JOA grading. According to their clinical symptoms and imaging findings, the patients were treated with different operative approaches and methods (posterior occipitocervical decompression and/or cistema magna reconstruction for atlanto-occipital malformation, and anterior cervical microdiscectomy with titanium intervertebralcage fusion or expansive open-door laminoplasty for cervical spondylotic myelopathy, and cavitary drainage for syringomyelia). All of the patients were observed by follow-up to evaluate their post-operative conditions. Results Of the 22 cases, the operative outcome was graded as excellent(difference between pre- and post-operative JOA scores was more than 2) in 6 cases, as good (the difference was 1) in 13, as unchanged (the difference was 0) in 3. In the follow-up of 2-48 months, 20 cases get further better, and 2 cases became stable. Conclusion The patients with atlanto-occipitalmalformation and cervical spondylotic myelopathy can be treated by one-stage operation with different approaches and methods according to their clinical manifestations and signs and imaging features. The treatment can reach excellent clinical and radiological outcomes and low morbidity.