Encephaloceles are pathological herniations of brain parenchyma through congenital or acquired osseus-dural defects of the skull base or cranial vault. Although encephaloceles are known as rare conditions, several surgical reports and clinical series focusing on spontaneous encephaloceles of the temporal lobe may be found in the otological, maxillofacial, radiological, and neurosurgical literature. A variety of symptoms such as occult or symptomatic CSF fistulas, recurrent meningitis, middle ear effusions or infections, conductive hearing loss, and medically intractable epilepsy have been described in patients harboring spontaneous encephaloceles of middle cranial fossa origin. Both open procedures and endoscopic techniques have been advocated for the treatment of such conditions. The authors discuss the pathogenesis, diagnostic assessment, and therapeutic management of spontaneous temporal lobe encephaloceles. Although diagnosis and treatment may differ on a case-by-case basis, review of the available literature suggests that spontaneous encephaloceles of middle cranial fossa origin are a more common pathology than previously believed. In particular, spontaneous cases of posteroinferior encephaloceles involving the tegmen tympani and the middle ear have been very well described in the medical literature.