Forty-two cases of pauci-immune necrotizing glomerulonephritis were reviewed on a 10 years period. Selection was exclusively based on histological criteria, i.e. at least one elementary lesion of extracapillary proliferation and/or glomerular necrosis, without immunoglobulin deposits. Mean age was 56. Thirty per cent of patients presented with normal or non-worsening renal function. At least one extra-renal sign was present in 66% of patients. ANCA were found in 9/20 cases. Death occurred in 12 patients. Age over 60 and oligo anuria were the most predictive factors for the fatal outcome. Half of the patients were on dialysis at the end of their follow-up. The renal function at presentation was the main predictive variable for renal outcome. Severe tubular necrosis was associated with a poorer renal evolution whereas treatment with cyclophosphamide led to frequent improvement in this retrospective study.