A 54-year-old woman had nausea, vomiting and personality changes since a few weeks. Paresis of the abducens and hypoglossal nerves on the left side, a right sided reduction of the visual field, discrete dysarthri×a and ataxia were diagnosed. Cranial magnetic resonance imaging demonstrated a contrast enhancing circular meningeal lesion of the foramen magnum. Histological examination revealed a granulomatous lesion of the meninges with focal necrosis, vasculitis and neutrophils indicating immune complex reactions. A diagnosis of primary meningeal Wegener's disease was made. Medication with low dose prednisolone led to complete remission of the lesion 1.5 years later.