Arteriovenous malformations (AVMs) are vascular lesions that are thought to arise from congenital errors during development of vessels resulting in abnormal connections between arteries and veins. Though most AVMs develop in the brain or spinal cord, they can occur anywhere in the body. These extracranial or peripheral AVMs have a predilection for the head, neck, and limbs. Since these malformations infiltrate normal soft tissue, management requires selective treatment with preservation of normal surrounding architecture. Therefore, they are best addressed through a staged multimodal and multidisciplinary approach, using a combination of different laser, interstitial, intravascular and surgical techniques to specifically target anomalous vessels. The goal of treatment is overall disease improvement and symptom control with interventions that do not result in outcomes worse than the disease itself. Recently, the discovery of somatic and germline mutations in peripheral AVMs have contributed to a better understanding of the pathophysiology, resulting in promising new pharmacologic treatments. Recent evidence suggests that adjuvant medical therapy can enhance and sustain interventional and/or surgical outcomes. Herein we describe how a new understanding of the etiology and physiology of extracranial AVM provides guidance to current treatment approaches.