Arteriovenous malformations (AVMs) are a group of high-flow congenital vascular malformations. They are characterized by abnormal shunting of the blood supply from fast-flow feeding arteries to low-resistance draining veins via a cluster of aberrant blood vessels termed a central nidus. They are often sporadic but can be associated with syndromes. AVMs are of the most challenging vascular malformations to diagnose and manage and often lead to significant morbidity and mortality. Early diagnosis by recognizing clinical features and experienced multidisciplinary team management is essential to minimize and avoid later complications. This article focuses on clinical findings and natural history of AVMs.