Four cases of suspected cerebral vasculitis have been described in association with acute posterior multifocal placoid pigment epitheliopathy, but none was confirmed histopathologically. We describe a patient who developed fatal cerebral vasculitis after placoid pigment epitheliopathy. Histopathologic data revealed a focal granulomatous inflammation of medium-sized arteries in the leptomeninges. Multinucleated giant cells were a conspicuous part of the inflammatory reaction. The cause of both placoid pigment epitheliopathy and cerebral vasculitis remains obscure.