Arteriovenous malformation (AVM) is a direct communication between an artery and vein without capillary connections and it is mainly found in the intracranial region. However, in an extracranial region, an AVM of the external ear is relatively uncommon. Recently, we experienced a case of an AVM in the external ear. A 20-year-old male patient presented with pulsatile tinnitus over the past 7 months and a reddish and pulsatile mass of the left external ear. We successfully treated the patient for the AVM in the external ear by complete excision after preoperative selective embolization.