Atlantoaxial 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. One patient was a 7-year-old boy who underwent tympanoplasty for cholesteatoma. The other patient was a 5-year-old girl with profound sensorineural hearing loss who underwent cochlear implantation. Both patients developed atlantoaxial rotatory fixation on the day after surgery, and they were treated conservatively. Our literature search using relevant terms identified 12 similar published cases. Thus, a total of 14 patients, including our 2 patients, were evaluated. Most of the patients were children and typically they complained of painful torticollis and exhibited a characteristic posture called the “cock-robin” position on the day after surgery. Mostly, the direction of torticollis was opposite to the side of surgery. Most of the patients received conservative treatment alone, but three underwent surgical treatment. The correlation between the direction of torticollis and the side of surgery suggests that rotation of the head during surgery has an impact on development of postoperative atlantoaxial rotatory fixation. Thus, children undergoing otologic surgery are thought to be at a risk of postoperative atlantoaxial rotatory fixation. Although rare, the surgical team needs to be aware of this adverse event and pay close attention to this possibility throughout the perioperative period. Perioperative management should include informed consent, preoperative assessment of the range of head and neck motion, proper intraoperative positioning and monitoring of the position, and postoperative follow-up. Postoperative atlantoaxial rotatory fixation is not completely preventable, but good perioperative management can minimize the damage resulting from this condition.