Aberrant internal carotid artery in the middle ear is rare and may be misdiagnosed, leading to severe complications. Indeed, approximately 1% of new cases are misdiagnosed. We present here a case of aberrant internal carotid artery with the symptoms of aural fullness, hearing impairment, and vertigo. The aetiology, clinical characteristics, and management of this case are discussed. Our experience illustrates that after confirmation of the diagnosis of aberrant internal carotid artery, localized treatment and/or surgical procedures should be avoided.