Study Design. A retrospective study. Objective. Investigate the diagnosis and surgery strategy for treatment of development spinal canal stenosis (DSSA) at atlas plane based on computerized tography (CT) image characters. Summary of Background Data. The occurrence of spinal canal stenosis in the atlas plane is relatively rare compared with lower cervical. Methods. Fifteen patients diagnosed with DSSA were included from 2014 to 2018. They are divided into four subgroups based on the character of CT images: group I (small size atlas), group II (hypertrophy of posterior arch of the atlas [PAA]), group III (incurved of PAA), and group IV (hypertrophy odontoid).Results. There are type I 7, type II 3, type III 2, and group IV 3 in the 15 cases. All the patients received different surgery procedures respectively: (1) posterior arch osteotomy were performed for group I/III//IV without atlantoaxial dislocation, (2) posterior arch resect and replantation were performed for group II, (3) occipital cervical fixation and fusion were added to the patients with associated atlantoaxial dislocation (AAD), ( 4) a new method of odontoid reduce and atlantoaxial fixation by transoral approach were performed for group IV with associated AAD. All cases underwent surgery successfully which included posterior occipitocervical fixation (OCF) þ posterior arch resection (PAR) eight cases, PAR four cases, posterior arch remodeling and re-implantation (PARR) two cases, and Dens remodeling þ trans-oral anterior reduction and plate fixation (DRþTARP) one case without severe complications. All patients show different improvement in the symptoms. Japanese orthopaedic association score improved from 9.2 to 14.7 in 1 year follow-up. Conclusion. DSSA could be easily diagnosed and divided into four subgroups according to the character of CT image, corresponding surgery strategy could receive a fine clinical result.