Introduction: Uretero-arterial fistula is a rare life-threatening condition. Its diagnosis and management remains a challenge for urologists. Case presentation: A 64-year-old man presented to our hospital with gross hematuria. He had history of rectal cancer treated with neoadjuvant chemoradiotherapy followed by low anterior resection and chronic ureteral stenting for bilateral ureteral strictures. He developed recurrent hemorrhagic shocks due to sudden massive gross hematuria. Repeated computed tomography and angiography could not identify the source of bleeding. After prophylactic embolization of the right renal artery and right nephrectomy, angiography finally revealed a uretero-arterial fistula from the right external iliac artery. Percutaneous balloon-expandable covered stent graft was used to successfully treat the fistula. Conclusions: Urologists should consider possible uretero-arterial fistula in patients with recurrent hematuria along with several risk factors and convincing medical history.