Arteriovenous malformations (AVMs) are congenital vascular anomalies, which seldom occur in the craniofacial regions. The rarity of these lesions compounds their extreme management difficulty. The clinical manifestation, vascular architecture, natural progress history and therapeutic strategy on AVMs in the craniofacial regions were reviewed in this paper. It is concluded that surgical ligation or occlusion of feeding arteries proves totally futile as the phenomenon of neovascular recruitment reconstituted arterial inflow to the AVM nidus. The procedure should be prohibited to do because of the ineffectiveness and hindering further endovascular treatment.