A middle aged man showed retroperitoneal fibrosis with signs of vasculitis verified by biopsy of the retroperitoneal mass. Antineutrophil cytoplasmic antibodies (ANCA) with cytoplasmic staining pattern (cANCA) were strongly positive, although there were no clinical or histological signs of Wegener's granulomatosis. No cases of cANCA associated retroperitoneal fibrosis have been described except in connection with Wegener's granulomatosis. ANCA have been found to associate with any inflammatory disorders. This observation may broaden the clinical spectrum of cANCA positive vasculitides.