Anti-glomerular basement membrane (anti-GBM) disease was usually described as a small vessel vasculitis presenting with acute kidney injury, haematuria and non-nephrotic proteinuria. We report a case of anti-GBM disease revealed by an intense nephrotic syndrome. The urinary protein level was 12g/day. Renal biopsy only showed crescent glomerulonephritis with linear staining of IgG in direct immunofluorescence without other glomerulonephritis. Immunoglobulin G (IgG) anti-GBM antibody titer was elevated.