Arteriovenous malformations (AVMs) are congenital vascular malformations (CVMs) resulting from birth defects of the vasculature. It is rarely seen, only accounting for 1.5% of all vascular anomalies, and 50% of the lesions are located in the oral and maxillofacial region. Regardless of the type, AVMs may ultimately lead to significant anatomical, pathophysiological and hemodynamic consequences. Therefore, despite their relative rarity, AVMs still remain the most challenging and/or life-threatening form of vascular anomalies. Transarterial coil embolisation or ligation of feeding arteries are incorrect approaches and may result in progress of the lesion. Furthermore, such procedures would prevent future endovascular access to the lesions via the arterial route, and should be abandoned. Interventional embolisation using various sclerosants is currently the mainstay of treatment for AVMs, and elimination of the nidus (if present) is the key to success. Among various embolosclerotherapy agents, ethanol sclerotherapy produces the best long-term outcomes, with minimal complications. For more complex cases, multidisciplinary approaches and interventions may provide an excellent potential for a curative result. Based on the published literature and clinical experiences, a practical treatment guideline was established in order to provide a criterion for the management of oral and maxillofacial AVMs. This protocol will be renewed and updated to reflect cutting edge knowledge, and provide the newest treatment modalities for oral and maxillofacial AVMs. Keywords: oral and maxillofacial region, arteriovenous malformations, treatment guideline