Meningoencephalic herniation into the middle ear is a rare and potentially life-threatening condition that may require prompt surgical intervention. Preoperative diagnosis is based on a high index of suspicion. Sometimes, however, meningoencephalic herniation is discovered during surgery. High-resolution computed tomography and magnetic resonance imaging should be performed to confirm the diagnosis and to evaluate the extension of the herniated tissue. This article discusses the diagnostic approach, management strategy, and surgical technique used in 27 patients with meningoencephalic herniation. In an attempt to avoid infective complications, the authors used the middle cranial approach in patients with large herniations.