Fibrous dysplasia is a benign disorder of bone primarily affecting young people from childhood through the third decade. It usually manifests as an enlargement of bones in and about the orbits or the the cranial vault. Whether localized or diffuse, it rarely involves the cervical spine. We report a case of fibrous dysplasia involving axis combined with atlantoaxial instability which was caused by odontoid process fracture. Transfacetal screw fixation augmented with Halifax interlaminar clamp technique was applied to obtain atlantoaxial stability. Postoperative course was unevenful and postoperative radiologic findings revealed favorable fixation of atlantoaxial joint. The pertinent literature on fibrous dysplasia and atlantoaxial instability is reviewed and discussed.