Coexistence of infrarenal aortic occlusion, known as Lerichesyndrome, and horseshoe kidney is extremely rare. Theisthmus of the latter, which usually contains functional renalparenchyma, complicates surgery. A 52-year-old male patientwith Leriche syndrome and concomitant horseshoe kidneydisease underwent a successful surgical repair. A medianincision and transperitoneal approach were used to explorethe infrarenal aorta, aortoiliac bifurcation, iliac arteries, andhorseshoe kidney. The isthmus was connecting the lower polesover the aortoiliac bifurcation. Aortoplasty with aortobifemoralbypass was performed, preserving the horseshoe kidney andits accessory arteries. Operative and postoperative periodswere uneventful. At eight months of follow-up, the patient wasasymptomatic in daily activities.