Cerebral arteriovenous malformations (cAVMs) are rare congenital anomalies of cerebral blood vessels that result from maldevelopment of the capillary bed, permitting direct communication between cerebral arteries and veins. It usually occurs in the supratentorial area of the brain; however, it can occur anywhere in the brain and spinal cord. Most of the patients with cAVMs present with a variety of complaints such as seizures, intracerebral hemorrhage, headache, and progressive focal neurological deficit. Imaging such as CT, MRI, and angiography plays a vital role in diagnosis, grading, risk assessment, and posttherapeutic follow-up. The multidisciplinary team use three therapeutic modalities in the treatment of cAVMs. This chapter reviews the clinical presentations, diagnosis, classification, and treatment of cAVMs.