Goodpasture s syndrome (GS) is an auto-immune disease that is part of the pulmonary-renal syndrome spectrum. It is characterized by the linear deposition of anti-glomerular basement membrane antibodies (anti-GBM) in glomerular and alveolar basement membrane resulting in alveolar hemorrhage and progressive glomerulonephritis. An early diagnosis is important to decrease clinical morbidity. In the present work we illustrate a GS case initially diagnosed as Wegener s granulomatosis. The patient showed favorable clinical evolution with corticosteroid therapy associated with plasmapheresis and cyclophosphamide.