Willam's syndrome, in order to more completely delineate the total spectrum of the disorder, indicates that"infantile hypercalcemia", "peculiar facies" and "supravalvular aortic stenosis." It has other many vascularanomalies, such as periheral pulmonary arterial stenosis, coronary arterial stenosis, celiac arterial stenosis,and renal arterial stenosis. Only 32% of the patients have evidence of supravalvular aortic stenosis. And it isvery rare disease entity that has been reported rarely in korea. Recently authers experienced a case that wasquestioned William's syndrome with peripheral pulmonary arterial stenosis, clinically and preliminaryradiologically and this case was confirmed by operation. Here we report a case of William's syndrome withperipheral pulmonary arterial stenosis and reviewed literatures.