OBJECTIVE To report a case of giant fibroepithelial polyp of the ureter and review the literature with special reference to the diagnosis and treatment of this benign condition. METHODS A young male patient with a nonfunctioning left kidney caused by a giant fibroepithelial polyp of the ureter is presented. The diagnostic methods and treatment options are discussed. RESULTS Following the diagnosis of obstructive uropathy with loss of left renal unit function, a left nephroureterectomy was performed which revealed a giant polypoid mass. CONCLUSION Fibroepithelial polyp of the ureter is a benign condition that is difficult to diagnose with current endoscopic techniques. Endoscopy should be the first therapeutic option if loss of renal function is not irreversible.