Horseshoe kidney is rare non-fatal congenital renal fusion anomalies. Incidence of horseshoe kidney is one in every 400 patients and it is more in males than females. Complications of horseshoe kidney include uretropelvic junction obstruction, urinary tract infections, stones and hydronephrosis. The occurrence of parenchymal disease is rare. There is no literature data of renal parenchymal disease in the horseshoe kidney. A case on grade-3 renal parenchymal disease in the horseshoe kidney is reported here, also patient was suspected with uremic encephalopathy.
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