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Granulomatosis with polyangiitis is a rare disease. The usual renal histology is extracapillary proliferative glomerulonephritis. The renal manifestations of vasculitides associated with Anti-Nutrophil Cytoplasmic Antibodies (ANCA) can take various clinical forms but are classically dominated by glomerular involvement.The aim of our work was to describe the epidemiological, clinico-biological, histological, therapeutic and evolutionary aspects of renal granulomatosis associated with ANCA vasculitis.