Primary central nervous system (CNS) vasculitis of childhood is a rare disorder. The most common signs and symptoms are acute severe headache and focal neurologic deficit. It should be suspected in children who have an acquired neurologic deficit that remains unexplained after an initial basic evaluation. Diagnosis usually depends on brain magnetic resonance imaging and conventional angiography of cerebral vasculature. Stenosis is the most common angiographic finding and it usually affects the middle cerebral artery and its branches. Anterior and posterior circulation is rarely involved.