Background Abdominal aortic aneurysm (AAA) with concomitant aorto-retroarotic left renal vein fistula (ALRVF) is an extremely rare clinical condition. With the recent development of endovascular techniques, repair of such conditions with a complete minimal invasive approach is now possible. We reported here a case of endovascular repair of AAA with concomitant ALRVF. Case Presentation A 62-year-old gentleman presenting with AAA and concomitant ALRVF underwent complete endovascular repair, including an endovascular aortic aneurysm repair (EVAR) with bifurcated aortic graft as well as embolization of the aneurysm sac and deployment of a covered stent in the left retroarotic renal vein to achieve sealing of the arterial-venous fistula. The patient required no blood transfusion and no ICU stay. He has been followed up closely for 4 years and has been well clinically. Aneurysm sac size has remained stable. Conclusions Endovascular repair can be a safe and reliable surgical alternative to treat AAA with concomitant ALRVF. But long-term follow up and more clinical data are required to verify the durability of endovascular repair for such conditions.