Sarcoid-like necrotizing granulomatosis is an exceptional condition characterized by lesions of epithelioid granulomatosis and signs of vasculitis. It usually involves the lungs and has a favourable prognosis. We report the case of a young woman with a systemic form of the disease which affected not only the lung, but also the eye and the central nervous system. Under corticosteroid therapy the pulmonary lesions and the biological inflammatory syndrome disappeared, but the brain lesions followed a course of their own despite the addition to steroid treatment of an immunosuppressive therapy. This case highlights the superiority of MRI over CT of the brain in the diagnosis and monitoring of brain lesions: CT became normal after corticosteroid therapy was initiated, where as the neurological signs became worse and the brain lesions progressed on MR images.