Arteriovenous malformation (AVM) is an uncommon vascular anomaly usually present in intracranial location, however may be present in other sites of the body, mostly in the head and neck region. The lesion may be present since birth or caused by trauma, but may become obvious during puberty or pregnancy. The diagnosis can be confirmed by selective angiography. Treatment usually includes super selective embolization followed by surgical excision within 48 h. We hereby present a rare case of a grotesquely disfiguring congenital auricular arteriovenous malformation in a 77-year-old elderly man, which has recently been complicated by ulceration, infection and hemorrhage in the past 6 months. The pinna showed a 15 cm x 8 cm pulsatile, erythematous, ulcerative hemorrhagic swelling which on angiography revealed tortuous superficial temporal and occipital arteries supplying the lesion. Complete auricular excision with split skin grafting was carried out. The option of preoperative embolization was not considered due to his renal dysfunction and the high dye load required for embolizing the fast flow arteriovenous malformation.