A patient with Behçet’s syndrome developed hematuria, proteinuria, and rapidly progressive, severe, renal failure. Renal biopsy confirmed the presence of crescentic, necrotizing glomerulonephritis. Treatment with intravenous corticosteroids and oral cyclophosphamide (CTX) failed to arrest the decline in renal function. Intravenous pulse CTX was administered monthly for four treatment cycles with consequent improvement and stabilization of renal function, albeit at a markedly depressed glomerular filtration rate. The literature of case reports concerning crescentic glomerulonephritis in Behçet’s syndrome is briefly reviewed, and fails to provide a uniformly accepted method of treating this rare complication. Intravenous CTX may prove to be another therapeutic option for rapidly progressive glomerulonephritis in the setting of Behçet’s syndrome.