CNS vascular malformation is an umbrella term that encompasses a wide variety of pathologies, with a wide range of therapeutic and diagnostic importance. This range spans lesions with a risk of devastating neurological compromise to lesions with a slow, static or benign course. Advances in neurovascular imaging along with increased utilization of these advances, have resulted in more frequent identification of these lesions. In this article, we provide an overview on definitions and classifications of CNS vascular malformations and outline the etiologic, diagnostic, prognostic, and therapeutic features for each entity. This review covers intracranial and spinal cord vascular malformations and discusses syndromes associated with CNS vascular malformations.