Atlanto-occipital dislocation is a devastating ligamentous injury that most often turns fatal. However, because of on-site resuscitation improvements, the emergency teams are increasingly dealing with this condition. We report a rare case of atlanto-occipital dislocation (AOD) in a surviving patient with more than one-year follow-up. The mechanism of injury appears to be an extreme hyperextension applied to the head. This injury occurs more frequently in children since they are anatomically predisposed (flat articulation between the occiput and the atlas, increased ligamentous laxity). The diagnosis should be suggested by severe neurological injury after high trauma but also post-traumatic cardiorespiratory deficit. There have been reports of atlanto-occipital dilocations without neurologic impairment. A radiographic examination must be performed and lateral cervical radiographs should be acquired. However, additional imaging with CT or MRI may be required to aid diagnosis of AOD in cases in which radiographic findings are equivocal. Once the diagnosis of AOD has been confirmed, an anatomical classification should be made according to the magnitude of displacement. Fatal lesions are of neurological and vascular origin and some authors advocate the systematic use of angiography. Consensus regarding the management of AOD in adults has been achieved. Occipito-cervical arthrodesis is the recommended treatment option. We advocate a two-stage surgery: the patient is initially fitted with a halo vest then occipitocervical fusion is performed. Surgical treatment should be combined with cardiorespiratory management. The emergency teams should get familiar with this injury since they will be increasingly confronted to it. Early recognition and standard appropriate management is essential to avoid delayed treatment and complications.