Primary angiitis of the central nervous system (PACNS) is a rare vasculitis limited to the leptomeningeal and intraparenchymal vessels. Histologically, the angiitis may be granulomatous with giant cells, necrotizing, or lymphocytic in character, and mixed morphologic types often occur. It is frequently fatal when the diagnosis is performed too late. We describe an autopsy case with multiple cerebral infarcts.