OBJECTIVE To retrospectively evaluate the epidemiological and morphological features and outcome of surgical treatment of incomplete ossification of the dorsal neural arch of the atlas (IODA) in dogs with atlantoaxial instability (AAI). ANIMALS 106 AAI-affected dogs that underwent ventral fixation of the atlantoaxial joint. PROCEDURES Medical records and CT images for each dog were reviewed. Dogs were allocated to 1 of 2 groups on the basis of the presence or absence of IODA or of dens abnormalities (DAs) in CT images. RESULTS Of the 106 dogs with AAI, 75 had and 31 did not have IODA; 70 had and 36 did not have DAs. Incomplete ossification was present in the cranialmost, central, or caudalmost portion of the dorsal neural arch of the atlas in 59, 39, and 28 dogs, respectively; 2 or 3 portions were affected in 29 and 11 dogs, respectively. The mean CT value (in Hounsfield units) for the midline of the dorsal neural arch of the atlas in dogs with IODA was significantly lower than that for the same site in the dogs without IODA. The mean age at surgery for dogs with central IODA was significantly higher than that of the non-IODA group. The severity of spinal cord injury before or after atlantoaxial ventral fixation did not differ between the IODA and non-IODA groups. CONCLUSIONS AND CLINICAL RELEVANCE Results indicated that concomitant DAs or IODA is common in dogs with AAI. In dogs with incomplete ossification in the central part of the dorsal neural arch of the atlas, surgical treatment of AAI generally occurs at a middle to advanced age.