Arteriovenous malformations (AVMs) are congenital vascular malformations (CVMs) that can occur anywhere in the body and are caused by defects in the development of arteries and veins, resulting in direct traffic between vessels of different sizes, or small, dysplastic vessels forming a primitive vascular network that cannot mature and develop into capillaries and are called anomalous vascular masses (nidus). It is characterized by a direct flow of blood into the venous system through different fistulas, via arterial structures, with fast flow rates and low resistance. The systematic classification developed by different expert groups (Hamburg classification, ISSVA classification, Schobinger classification and angiographic classification) has led to a better understanding of the biology and natural course of AVM and to a significant improvement in the outcome of the treatment. the Hamburg classification is based on embryologic development and is divided into 2 categories: extra-trunk and trunk, which helps to determine the development of the lesion and the possibility of recurrence. Most AVMs are extrasegmental and have the potential for continued proliferation, while trunk AVMs are very rare. Regardless of the type, arteriovenous shunts eventually lead to severe anatomic, pathophysiologic, and hemodynamic changes. AVMs can be classified as arteriovenous fistulas (AVF, type I), anomalous vascular masses (nidus, type II), or venous aneurysmal dilatation (venous aneurysmal dilatation with multiple supply arteries and thickening of a single returning vein, type IIIa; venous aneurysmal dilatation with multiple supply arteries and thickening of multiple returning veins, type IIIb). Although rare (10% to 20% of CVM), AVMs remain the most challenging, life-threatening, or disabling vascular malformations. The clinical presentation of AVMs depends on the extent and size of the lesion and can be asymptomatic birthmarks or associated with congestive heart failure. Initial diagnosis and evaluation can be based on noninvasive or minimally invasive tests such as dual-function ultrasound, MRI, MRA, CT, and CTA. arteriography is the gold standard for the diagnosis of AVMs and is necessary to develop subsequent treatment. Multidisciplinary team management should combine surgical and non-surgical interventions for optimal outcomes. Currently available treatments carry a high risk of complications, but if the benefits are assessed to outweigh the harms, early and aggressive treatment should be given to eliminate the abnormal vascular mass, if any. Transarterial spring coil embolization or ligation of the supplying artery is the wrong treatment; the abnormal vascular mass remains intact and can lead to aggravation of the lesion development, and this treatment can prevent further interventions via the transarterial route. Surgically inoperable, infiltrative extratentorial AVM can be treated with endovascular intervention alone. Of the various embolization treatments, anhydrous ethanol embolization provides the best long-term outcome with the least recurrence, but the operator requires systematic training and sufficient experience to minimize complications. For surgically resectable lesions, surgical treatment can be used to obtain good control of the lesion. Preoperative embolization reduces intraoperative bleeding, shrinks the lesion and defines the lesion border, facilitating surgical resection, and this combined treatment is expected to yield the best curative results.