This study is aimed to report the largest independent case series of spontaneous otogenic pneumocephalus (SOP) and review its pathophysiology, clinical presentation, and treatment. Four patients underwent a middle cranial fossa approach for repair of the tegmen tympani and tegmen mastoideum. A comprehensive review of the literature regarding this disease entity was performed. U.S. tertiary academic medical center. Patients presenting to the lead author's clinic or to the emergency department with radiographic evidence of SOP. Symptoms included headache, otalgia, and neurologic deficits. Patients were assessed for length of stay, postoperative length of stay, and neurologic outcome. Three of four patients returned to their neurologic baseline following repair. Four patients were successfully managed via a middle cranial fossa approach to repairing the tegmen mastoideum. The middle cranial fossa approach is an effective strategy to repair defects of the tegmen mastoideum. SOP remains a clinically rare disease, with little published information on its diagnosis and treatment.