This study states that Lingual arteriovenous malformations (AVMs) are extremely rare in clinical practice, which has limited comprehensive research to find standard treatment protocols. This study summarizes the clinical features of lingual AVMs and assesses the safety and efficacy of ethanol embolotherapy in the management of these lesions. Our study group was composed of 52 patients with lingual AVMs treated by ethanol embolization, all of whom received general anesthesia. The optimal access to the nidus of the AVM was by direct puncture, transarterial catheterization, transvenous catheterization, and a combination of these routes. Pure ethanol was manually injected into the nidus of the AVMs. The observed major or minor complications related to ethanol embolization were analyzed, and periodic follow-up of the patients was performed. The devascularization of the lingual AVMs between baseline and final angiography and the clinical outcomes of symptoms and signs after ethanol embolization were evaluated. There were 171 embolization procedures (mean, 3.3; range, 1-20) including 166 ethanol embolizations performed; the average volume of ethanol injected in a single ethanol embolization session was 29.8 mL (range, 1-65 mL). Therapeutic outcomes were complete response in 17 patients (33%), partial response in 33 patients (63%), and no response in 2 patients (4%).