Spontaneous otogenic pneumocephalus caused by barotrauma is extremely rare. We surveyed the literature and describe a case report of otogenic pneumocephalus from barotrauma managed with a middle fossa craniotomy. The neurotologic literature review revealed three similar cases. We conclude that anatomic defects in the floor of the middle cranial fossa contribute to the development of spontaneous otogenic pneumocephalus. Neurotologic consultation should be obtained. In selected instances, middle fossa craniotomy may be required for repair.