Objectives Multiple organ failure is a rare manifestation of ilio-iliac arteriovenous fistula which can lead to a high rate of misdiagnosis and death. Methods We reported a 61-year-old man presenting with multiple organ failure rapidly after right lower limb swelling. Computed tomography angiography showed an ilio-iliac arteriovenous fistula caused by right common iliac artery aneurysm, and venous thrombosis of bilateral common iliac veins. A bifurcated stent-graft with coil embolization of right internal iliac artery was used for repair. Results The patient recovered rapidly and was discharged without complications. Although arteriovenous fistula persisted due to type II endoleak, aneurysm sac and inferior vena cava significantly shrunk at six months follow-up. Conclusions This report demonstrated that multiple organ failure may appear when the distal outflow tracts of arteriovenous fistula are obstructed. Moreover, endovascular repair is effective for reversal of multiple organ failure caused by arteriovenous fistula, even if arteriovenous fistula persists due to type II endoleak.