Objective: To evaluate the long-term outcome of endovascular and open treatment for nonatherosclerotic renal artery disease (NARAD). Design: Retrospective review. Setting: Academic institution. Patients: Fifty-five patients (47 women; mean age, 40 years) with NARAD. Underlying disease included Takayasu arteritis in 31 and fibromuscular dysplasia in 24. Interventions: Open revascularization and renal artery percutaneous transluminal angioplasty with or without stenting. Main Outcome Measures: Primary, primary assisted, and secondary patency rates; blood pressure; antihypertensive medication requirements; renal function; and mortality. Results: Seventy-nine renal interventions were performed, including 59 aortorenal bypass (16 ex vivo), 3 visceral-renal bypass, 12 endovascular (8 percutaneous transluminal angioplasty and 4 stent placements) procedures, and 5 nephrectomies. There were no in-hospital deaths. During a mean follow-up of 75 months, 1-, 3-, and 5-year primary patency rates for any intervention were 87%, 75%, and 75%, respectively; primary assisted/secondary patency rates were 92%, 86%, and 86%, respectively. Endovascular interventions at 1, 3, and 5 years had primary patency rates of 73%, 49%, and 49%, respectively, and primary assisted/secondary patency rates of 83%, 83%, and 83%, respectively. For open revascularization, 1-, 3-, and 5-year primary patency rates were 91%, 80%, and 80%, respectively; primary assisted/secondary patency rates were 94%, 87%, and 87%, respectively. For both interventions, blood pressure and the number of antihypertensives used were reduced compared with preintervention values (all P<.05). Serum creatinine level and estimated glomerular filtration rate were also improved after revascularization (both P<.05). There were 6 deaths. Five- and 10-year actuarial survival rates were 94% and 78%, respectively. Conclusions: Endovascular and open management of NARAD confers long-term benefit for blood pressure, renal function, renal artery/graft patency, and survival. Open revascularization results in superior 1- and 5-year outcomes compared with endovascular management and provides the most durable outcome for NARAD.