A 2-year-old girl who had been followed because of pulmonary valve stenosis since birth, underwent unsuccessful balloon valvuloplasty twice. The angiographic study showed thin cusps and remarkably thick and immobile filling defects in the main pulmonary artery, which indicated supravalvular PS or pulmonary valve dysplasia. Operation was done under conditions of a beating heart and total cardiopulmonary bypass. Pulmonary valve dysplasia was localized on the edge of all three cusps. After resection of the dysplastic lesion, the stenosis was released and slight regurgitation was observed by ultrasonography study.