Abstract: During routine dissection of a male cadaver in the anatomy unit, AIMST University, Malaysia, a solid mass was observed in the lower right and left lumbar region. Further fine dissection mass it was found to be a horseshoe shaped kidney. The both kidneys were found to be fused at the lower end by isthmus. The right kidney and left kidney are supplied by right and left renal arteries. Further, noticed that the accessory arteries arising from abdominal aorta and supplying to both kidney. The both lower poles of kidney connecting isthmus were supplied by a different artery taking origin from abdominal aorta. Ureters ran downwards anterior to isthmus. The inferior mesenteric artery ran over the mass of tissue joining the lower poles of two kidneys. The congenital malformation can be asymptomatic or can predispose the patient to ureteric obstruction, infection, hydronephrosis, malignancies and posing technical difficulties during renal surgeries, endovascular procedures, renal transplants.