The aim of this study was to report an experience with 190 cases of os odontoideum over 20 years. The management outcome following atlantoaxial fixation was analyzed. From January 2000 to September 2018, 190 patients with os odontoideum were surgically treated. There were 113 male patients and 77 female patients; average age was 24 years (range, 2-68 years).The patients were divided into 3 groups depending on the nature of atlantoaxial dislocation (group 1, mobile and partially or completely reducible atlantoaxial dislocation; group 2, fixed or irreducible atlantoaxial dislocation; group 3, presence of basilar invagination). There were 65 pediatric patients (<18 years old). All patients underwent atlantoaxial joint manipulation and lateral mass plate and screw fixation. The goal of surgery was segmental atlantoaxial arthrodesis. No transoral or posterior foramen magnum bone decompression was done. Occipital bone was not included in the fixation construct. On direct bone handling and observation, atlantoaxial joint pathologic hyperactivity related instability was identified in all patients. Atlantoaxial segmental stabilization resulted in clinical symptomatic and neurologic improvement in 100% of patients. Os odontoideum signifies chronic or long-standing atlantoaxial instability. Segmental atlantoaxial fixation is a reliable form of surgical treatment. Bone decompression is not necessary. Inclusion of occipital bone and subaxial vertebrae in the fixation construct is not necessary.