Objectives To review the diagnosis and treatment of ureteroarterial fistulas and to develop a management algorithm. Long-term ureteral stenting, along with pelvic surgery and radiotherapy, have led to an increased incidence of ureteroarterial fistulas. Experience diagnosing and treating these fistulas has been limited to case reports. Methods A retrospective chart review from 1975 to 2004 revealed eight ureteroarterial fistulas in 7 patients at our institution. The patient demographics, presenting symptoms, and diagnostic studies were reviewed. Also, we analyzed the treatment, hospital course, and long-term outcomes. Results Ureteroarterial fistulas were more common in women (86%) than in men. All patients presented with gross hematuria, and 57% had lateralizing flank pain. Risk factors included chronic indwelling stents (87%), previous pelvic external beam radiotherapy (71%), pelvic surgery (100%), and vascular disease (87%). Provocative angiography was diagnostic in only 63% of cases. Although not sensitive, cystoscopy revealed lateralized, pulsatile hematuria in all cases when performed. Treatment ranged from endovascular stenting with nephrostomy tube to primary surgical repair with nephrectomy. Conclusions On the basis of this review, we propose a systematic diagnostic and treatment approach to a serious disease process. The use of these proposed algorithms will minimize unnecessary testing, increase the speed of diagnosis, and potentially improve overall outcomes of patients with ureteroarterial fistulas.