Anti-glomerular basement membrane (anti-GBM) disease is an aggressive small vessel vasculitis usually mediated by IgG autoantibodies. We describe the case of a 73-year-old male with rapidly progressive renal failure that was diagnosed with IgA mediated anti-GBM disease associated with circulating anti-neutrophil cytoplasm antibodies (ANCA), where the diagnosis was established on kidney biopsy by detecting linear deposition of IgA along the GBM on immunofluorescence microscopy. Despite an intensive immunosuppressive regimen with plasmapheresis, steroids and oral cyclophosphamide, the disease progressed to end-stage renal failure and the patient was started on hemodialysis.