Extracranial arteriovenous malformation (AVM) is a high-flow congenital vascular malformation, where direct communication between the arteries and veins impedes perfusion of capillary beds and causes disfigurement of the affected tissue. Surgery and endovascular therapy are currently the main treatment for extracranial AVMs. Nevertheless, management of complex cases is sometimes challenging because of severe complications such as refractory ulceration, life-threatening bleeding, and even cardiac insufficiency. Here, we reviewed the development and potential treatment for extracranial AVMs and shared our single-center experiences of diagnosis and treatment of this challenging disease.