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The authors present the case of a patient with ischemic episodes and recurrent intracerebral hemorrhages probably caused by primary angiitis of the central nervous system (CNS). An initial angiogram revealed multiple cerebral artery aneurysms as well as vascular wall irregularity; a second angiogram obtained 2 years later, however, did not demonstrate the previous aneurysms but instead showed new ones together with stenosis. Based on the histopathological findings and clinical course in this case, the multiple aneurysms appear to have been induced by vascular wall fragility and subsequent self-repair resulting from primary angiitis of the CNS. The authors present the histological and clinical characteristics of this unusual case of granulomatous, necrotizing CNS vasculitis.