Arteriovenous malformations (AVMs) are vascular malformations that present high-flow direct communication between the arteries and veins, not involving the capillary beds. They can be progressive and lead to various manifestations, including abnormal skin or mucosal findings, ischemia, hemorrhage, and high-output heart failure in severe cases. AVMs often involve the head and neck region. Head and neck AVMs can present region-specific clinical manifestations, angioarchitecture, and complications, especially in cosmetic appearance and ingestion, respiratory, and neuronal functions. Therefore, when planning endovascular treatment of head and neck AVMs, physicians should consider not only the treatment strategy but also the preservation of the cosmetic appearance and critical functions. Knowledge of the functional vascular anatomy as well as treatment techniques should facilitate a successful management. This review summarizes AVMs' clinical manifestations, imaging findings, treatment strategy, and complications.