Angiitis signifies an inflammation of the blood vessels that can cause damage to vessel walls, vascular occlusion, and ischemic injury to organs served by these vessels, and it can occur in the central nervous system. All central nervous system vasculitides but one have been linked to the presence of various underlying disorders. This review covers primary and spinal cord granulomatous angiitis of the central nervous system, as well as secondary angiitis of the central nervous system and its associated underlying disorders.