Behçet's disease (BD) is a multisystem disorder of unknown etiology. Renal involvement in Behçet's disease is not frequent. Pauciimmune necrotizing and crescentic glomerulonephritis is associated with antineutrophil cytoplasmic antibodies (ANCA) which are believed to activate neutrophils and cause vasculitis, especially if there is a concurrent synergistic inflammatory process. In this case report, we describe a patient diagnosed with BD who also developed pauciimmune necrotizing and crescentic glomerulonephritis. We also compared this case with similar previous cases reported in the literature. The aim of this study is to add information regarding ANCA, pauciimmune glomerulonephritis and Behçet's disease.