We report a rare case of aberrant internal carotid artery in the middle ear. The patient was a 30-year-old man with pulsatile tinnitus. MRA and 3D-CTA revealed an aberrant course of the internal carotid artery in the middle ear. 3D-CTA proved useful for radiographic diagnosis, leading to the observation of an aberrant internal carotid artery passing the foramen. We discuss the diagnosis and management of such patients.