BackgroundAtlantoaxial rotatory fixation is a condition in which the first and second vertebrae of the cervical spine become interlocked in a rotated position. This condition can result in serious consequences and thus have a significant impact on patients, especially when diagnosis and treatment are delayed. Some cases of atlantoaxial rotatory fixation have been described in association with otologic surgery or plastic surgery involving the ear. We present the cases of two pediatric patients who developed atlantoaxial rotatory fixation following otologic surgery and we review the relevant literature.