Survival after traumatic atlantooccipital dislocation is rare. Only long-term survivors have been reported in the literature; however, improved prehospital care is likely responsible for the increase in the number of these patients seen at neurotrauma centers over the last decade. Associated severe and persistent neurological deficits are common in the few survivors. We report the case of a 10-year-old boy with traumatic atlantooccipital dislocation and a severe neurological injury. Low-field magnetic resonance imaging provided the additional diagnosis of an associated cervicomedullary epidural hematoma. The patient underwent emergency operative evacuation of the hematoma and an occipital-cervical fusion with internal fixation. He had a remarkable recovery in neurological function and achieved stable bony fusion 3 months postoperatively. With early recognition of this entity, improved neuroradiological imaging techniques, and aggressive treatment, patients may survive with significant neurological recovery.