RESUMO
Primary angiitis of the central nervous system (PACNS) is a rare idiopathic disorder with recurrent vasculitis restricted to the central nervous system (CNS) and involves small-sized (and at time medium-sized) blood vessels. The typical clinical manifestations include multifocal strokes or encephalopathy, often associated with severe headaches. There are very few pediatric cases with PACNS reported in the literature. The diagnosis of PACNS is mainly based on its clinical features, demonstration of angiitis on angiography or biopsy, and exclusion of secondary causes of angiitis. A high index of suspicion is necessary for the diagnosis. Though the prognosis is generally poor, use of steroids and immunosuppressive therapy has been useful in achieving successful remission in many patients. We report the case of a patient with PACNS presenting recurrent headaches for 5 days.