Arteriovenous malformations (AVMs) are a rare congenital vascular disorder. They represent a fast-flow vascular malformation. Clinically, AVMs present a heterogenous expression and can affect every part of the body. Here, we will solely focus on extracranial AVMs. Generally, AVMs progress with the patient's age. Patients often suffer from pulsation, skin discoloration, pain, ulceration, bleeding, and disfigurement. Diagnostic tools include color-coded duplex sonography, MRI and CT imaging, as well as the clinical examination. 4D dynamic perfusion-computed tomography may help in the interventional planning. Digital subtraction angiography is required during interventional therapy. AVMs pose a great challenge to the treating physician. The therapy of this rare disease should be managed in an interdisciplinary center for vascular malformations. It consists of conservative measures, such as compression garments and pain medication, transcatheter or, more rarely, percutanous embolization, and surgical resection. In smaller, localized lesions, resection with primary wound closure may be feasible, whereas extensive AVMs regularly require the reconstruction of the resulting soft tissue defect and possibly affected functional structures by means of free tissue transfer. In the interdisciplinary setting required for an appropriate treatment of AVMs, extensive knowledge of the various therapies, including those from different specialties, is necessary. Therefore, this article aims to provide an overview over both the interventional and surgical therapeutic options.