Horseshoe kidney is the commonest congenital renal anomaly. Its inherently-poor drainage system renders the patient prone to stone formation. A 56-year-old man with bilateral renal stones in a horseshoe system associated with hydronephrosis is presented. He was treated successfully with bilateral CT-guided percutaneous nephrostomy and percutaneous nephrolithotripsy. Various treatment options, including ESWL, PCNL and open surgery, are discussed.