The horseshoe kidney (HSK) is a fusion anomaly of the kidneys with an incidence of 0.25% in general population, being more prevalent in men than women in a 2:1 relation. [1] This abnormality has been discovered clinically in all age groups ranging from fetal life to 80 years, but in autopsy series it is more prevalent in children. [2] This early age prevalence has been explained by high incidence of multiple congenital anomalies associated with the HSK, some of which are incompatible with longterm survival. [3] There is a wide variation in HSK vascular supply and excretory system. Renal arteries can originate from the abdominal aorta (Ao), the iliac arteries, and the inferior mesenteric arteries. Therefore it is important to know the clinical and surgical anatomy of the HSK. Renal transplants with HSK have been performed and for the proper management of this clinical condition it is necessary to understand the surgical difficulties and the most frequent clinical complications such as urolithiasis.In view of this important kidney anomaly, we report a case with a unusual arterial supply.