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Multicystic dysplastic kidney disorder (MCDK) is commonly sporadic but is rarely an inherited congenital disease. It is the commonest cause of abnormally enlarged kidney, diagnosed on antenatal ultrasound examination. It is typically a unilateral disorder; bilateral condition is incompatible with extra uterine life. Association of this disease with abnormalities of various organs is common . We report a rare case of bilateral multicystic dysplastic kidney associated with high ano-rectal anomaly which was diagnosed antenatally and pregnancy was terminated.