Atlantoaxial rotatory subluxation (AARS) is an infrequent condition that occurs most commonly in children for unknown reasons. Pediatric surgery, otopharyngeal inflammation, general anesthesia, and extreme rotation of the head are risk factors for development of postsurgical AARS, but AARS can often occur unnoticed, and the syndrome is not well known. We encountered three cases of postoperative AARS that occurred within 7 months; therefore, we have developed guidelines for prevention and early treatment of postoperative AARS. Postoperative AARS cannot be eliminated completely, but informed consent, a preoperative check, an appropriate surgical position, and a postoperative check may reduce the risk and damage related to this condition.