Although aortic and pulmonary valve stenosis are among the most common congenital heart defects, the combination of both aortic and pulmonary valve stenosis in the same patient appears to be very uncommon. Accurate diagnosis of combined valvular stenosis is imperative prior to surgical correction, otherwise surgery of one of the lesions may result in an insufficient hemodynamic improvement. A seven-year-old girl with congenital aortic and pulmonary valve stenosis associated with hypoplastic aortic annulus underwent Konno's operation, pulmonary valvotomy and resection of anomalous muscle of the right ventricular outflow tract simultaneously. The operation was successfully performed and postoperative course was uneventful.