Aneurysm may be classified into intracranial and extracranial according to its location. Extracranial aneurysm is relatively uncommon compared to intracranial aneurysm and it is rare in the middle ear cavity. The causes of extracranial aneurysm include atherosclerosis, dysplasia, blunt or penetrating trauma, local radiotherapy, acute dissection and infective lesions. Among the extracranial aneurysm, it is reported that carotid aneurysm in the temporal bone may be confused with glomus body tumor, which is usually larger. A typical presentation is pulsatile mass and it may accompany neurologic symptoms. We report a case with a review of literature of an external carotid aneurysm in the jugular fossa and middle ear cavity.