Angiitis of the central nervous system (CNS) remains a poorly understood and clinically challenging form of vascular inflammatory disease. Primary angiitis of the CNS (PACNS) has been viewed as a relentless and uniformly fatal disorder if untreated. In addition, recent trends have demonstrated an increasing reliance on angiographic diagnosis without tissue confirmation. It has been suggested that PACNS is clinically more heterogeneous than previously appreciated and may include relatively benign subsets. A reappraisal of diagnostic approaches has suggested caution in the diagnosis of CNS angiitis on purely angiographic grounds. Secondary vasculitis of the CNS is even more heterogeneous. Clinicians involved in the evaluation of patients with presumed CNS vasculitis need to be aware of the clinical spectrum of vascular inflammatory disease within the CNS as well as the strengths and limitations of currently available diagnostic modalities.