A horseshoe kidney poses special problems during treatment of an abdominal aortic aneurysm (AAA), and there is much controversy about the most suitable method of repair. We report the case of a 65-year-old man with a horseshoe kidney, in whom an AAA was treated with a unibody bifurcated endograft. During the procedure, the two anomalous renal arteries were sacrificed. Although there was transient elevation of the creatinine levels, the patient was discharged with normal renal function and no endoleak from the accessory renal arteries. Thus, when two normal and two accessory renal arteries arise from the non-aneurysmatic proximal aortic neck, providing that preoperative kidney function is normal, it seems that the treatment can be safely carried out using an endovascular technique and excluding the accessory renal arteries. This case supports the feasibility of endovascular surgery for the treatment of AAA in the presence of a horseshoe kidney.