Brain herniation into the middle ear is very rarely seen. In addition to reasons like congenital factors, trauma, and infection, tegmen defect may develop as a result of iatrogenic events secondary to chronic otitis media surgery with or without cholesteatoma. Since it may cause life-threatening complications, patients must be evaluated and monitored for tegmen defect. In this paper, diagnosis and treatment of a brain herniation case due to iatrogenic tegmen defect were described along with relevant literature.