Background: Traumatic atlanto-occipital dislocation (AOD) is a rare but often fatal injury. Consequently, long-term data regarding surviving patients have been limited. In particular, the occurrence of hydrocephalus is not well-documented. Case Description: A 33-year-old male sustained AOD as a consequence of a motor vehicle collision. Although he did well initially after an occipitocervical fusion, 1 month after his operation, he exhibited signs of increased intracranial pressure (bilateral abducens nerve palsies, worsening headaches, and fatigue). He was found to have hydrocephalus, which was responsive to shunting. Conclusion: Manifestations of hydrocephalus after AOD can be variable, ranging from interval ventricular dilatation to pseudomeningoceles and syringomyelia. In addition, the timing of presentation can be acute, requiring emergent external ventricular drainage, or delayed, requiring ongoing vigilance. Consequently, as more patients survive this once thought to be fatal injury, caution for hydrocephalus is stressed.