Iliac vein-ureteral fistula is a rare cause of hematuria. The diagnosis of an iliac vein-ureteral fistula can be elusive even with the use of multiple methods. With regards to the treatment, there appears to be a shift in management from primarily open surgical to primarily angiographic management. We present a unique case of an external iliac vein - transplant ureteral fistula. A 48 year-old female complained of recurrent gross hematuria. She underwent transplant nephrectomy and radical left nephrectomy because of rejection of transplanted kidney and cystic renal cell carcinoma when the hematuria arose for the first time. Ten months later, the hematuria recurred again, and cystoscopy showed bleeding from the right transplant ureteral orifice. Open exploration confirmed the diagnosis of external iliac vein - transplant ureteral fistula. Diagnostic difficulties and treatment dilemma of such a rare cause of hematuria are also discussed.