Arterio-ureteral fistula (AUF) is a rare, but potentially lethal, clinical entity. In spite of an increase in reported cases and clinical awareness, AUF is not always recognized in time. Delay between first presentation and treatment may adversely affect clinical outcome. What We Found: In this systematic review we found 245 published articles with 445 patients and 470 AUFs. Most patients had a medical history of chronic indwelling ureteral stents (80%), pelvic oncology (70%), irradiation (53%) or vascular surgery (26%), presenting with intermittent (micro) hematuria or building up to massive hematuria. The pathophysiologic hypothesis is presented in the figure . AUF was located at the crossing between ureter and artery, mostly the common iliac artery. The best modality to diagnose this entity was an angiography, with a sensitivity of 62%. Endovascular stent graft placement is preferred over open surgical repair in terms of AUF-related mortality (4% vs 11%). AUF-specific mortality before 2000 vs after 2000 is 19% vs 7%, coinciding with increasing use of endovascular stent graft placement.Limitations: The retrospective nature of this study, including many single case reports, results in a low level of evidence and high risk of bias. Furthermore, report bias could impact our findings such as better medical registration, electronic patient files, better imaging tests, less invasive treatment options, and better outcome. Interpretation for Patient Care: Clinical awareness as well as multidisciplinary approach is important to decrease unnecessary delay between first presentation and treatment of AUF. The best diagnostic tool is an angiography and the preferred treatment is endovascular stent graft placement.