Small artery vasculitis of the CNS is a rare and serious condition characterized by the inflammation of blood vessels within the brain and spinal cord. There are two groups of small artery vasculitis determined by the presence or absence of immunoglobulin complex deposition in the vessel wall. ⋯ Absence of immune complex deposition is associated with anti-neutrophil cytoplasmic antibody (ANCA) and includes microscopic polyangiitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and primary angiitis of the CNS. These conditions present a diagnostic challenge in which imaging plays a crucial role.