Objective: To describe a horseshoe kidney, a congenital anomaly of the upper urinary tract. Clinical Presentation: A case study of horseshoe kidney harvested from a 62-year-old cadaver at Gazi University Medical School is presented. Results: The right and left kidneys were fused at their lower poles by a parenchymal isthmus located ventral to the abdominal aorta and formed a U-shape with two unequal arms. The isthmus of the ectopic kidney was placed obliquely to the left at the level of the fourth and fifth lumbar vertebra. The left kidney was larger and longer than the right one. The kidneys were supplied by three renal arteries arising from the abdominal aorta. Two arteries on the right side supplied blood of the two kidneys, while the third artery that directly originated from the aorta, above the origin of inferior mesenteric artery, supplied the isthmus. Venous drainage of the both kidneys and the isthmus were drained by three veins that opened independently into the inferior vena cava. The right ureter was duplicated in origin. Conclusion: This report shows that knowledge of anomalies such as this is very important in planning and conducting surgical procedures. Copyright (c) 2007 S. Karger AG, Basel.