OBJECTIVE Concomitant atlantoaxial and atlanto-occipital subluxation resulting from any cause is extremely rare. We have found only five previously reported cases and describe another, suggesting a treatment plan. METHODS A 13-year-old female patient presented with a 3-month history of neck pain and decreased neck movements. All symptoms started after a localized neck infection had been treated successfully with antibiotics. There was no history of trauma. A diagnosis of postinfective atlanto-occipital and atlantoaxial rotatory subluxation was made based on a plain roentgenogram and was confirmed based on a computed tomographic scan. METHODS The atlantoaxial and atlanto-occipital subluxation was reduced during surgery. A posterior C1-C2 fixation was performed, and the atlanto-occipital joint was stabilized by means of a halo body jacket for 3 months. One year after removal of the jacket, all subluxation remained reduced and the patient retained significant neck movement. CONCLUSIONS Disruption of the occipito-atlanto-axial complex can result from relatively minor head and neck infections and should be suspected in children with persisting neck pain and decreased neck movements. It may not be necessary to perform an occipitoaxial fusion to treat these patients, and a more limited fusion may be successful.