Tumors arising within horseshoe kidney are uncommon and may be difficult to identify. The incidence of tumor within horseshoe kidney is higher than in the normal one. Adenocarcinoma is the most common tumor. The nephrectomy is considered as partial and the patients need a strict follow up. Horseshoe kidneys show a lot of variety with regard to supporting arteries and veins. Therefore, a selective study of the renal vessels should be performed preoperatively. Abdominal angiography should be done to study this vasculature.