Objective: Arteriovenous malformations (AVM) of the ear show unique features. Progressive growth or inadequate management of AVMs may lead to bleeding, infection, cartilage exposure, and ultimately loss of structure. Total ear amputation is an alternative; however, due to the structural complexity of the ear, reconstruction is technically challenging. This study presents a treatment algorithm based on a clinical series, with treatment options including resection and immediate and late reconstruction.Methods: Between 2004 and 2018, 12 patients with auricular AVMs were treated with AVM resection and ear reconstruction at our hospital. Among these 12 patients, seven were women, and patients' ages ranged between 4 and 34 years. Parameters considered before resection and reconstruction were compromised ear extension (partial, total, or extra-auricular involvement), thickness (cutaneous and cartilaginous), symptoms (bleeding, infection, ulceration, and/or cartilage exposure), and preoperative embolization. Total resection of AVMs was planned as the first step in all cases, followed by primary closure. AVM resection was categorized into a partial or total procedure, resulting in partial or total ear defects. Reconstruction was categorized as immediate or delayed.Results: Preoperative embolization was performed in 10 patients. AVMs were totally removed, resulting in seven total ear amputations. In six of these patients, total ear reconstruction was performed 6 months later, and all patients were recurrence-free. Only one patient did not undergo ear reconstruction. Regrowth was not observed in any reconstructed ear.Conclusion: Treatment of ear AVMs requires a planned approach to achieve favorable clinical outcomes and for concomitant safe and definitive reconstruction.