Objective · To study the application of occipitocervical fusion and fixation in malformation of craniocervical junction surgery.   Methods · Thirty-six consecutive patients with craniocervical junction malformation were decompressed under evoked-potential monitoring. Sixteen  patients were treated with posterior occipitocervical fusion and fixation using Vertex screw-hook system, and 11 using Vertex screw-rod system, other  patients using Mountaineer OCT spinal system. All patients were followed up for 2 to 12 years (mean 7 years). The recovery rate was analysed based  on the scoring system of the Japanese Orthopaedic Association (JOA).  Results · All the patients were followed up. A stable bony fusion according to  radiological criteria was achieved in all cases. There was no implant broken and dislocation. The improvement rate according to JOA scoring system  were evaluated. Twenty-four cases (66.7%) got cured; 4 cases (11.1%) had remarkable effects; 8 cases (22.2%) were effective; none was ineffective.   Conclusion · Occipitocervical stabilizations hope to be restored through occipitocervical fusion and fixation. Good results can be obtained in most patients  with complete radiological data, proper surgery indication, suitable surgical modality and internal fixation materials.