This paper presents a case report of a fourteen-year-old girl with a cervicooculo-acusticus syndrome. The cervical anomalies consisted of occipito-cervical fusion with accessory occipital vertebrae and a typical Klippel-Feil anomaly of the lower cervical spine. Sudden hyperextension of the neck appeared to concentrate stress at the most mobile segment of the neck—between the atlas and axis. The resultant epidural hematoma compressed the cervical cord at that level and caused her death.