Keimyung Medical Journal, Vol.6(2) : 405-410, 1987
Williams syndrome is an uncommon congenital anomaly which is composed of supravalvar aortic stenosis, peculiar face (elfin facies) and mental retardation. Authors experienced a case of Williams syndrome in a 7 year old girl who had a peculiar face and mental retardation. Diagnosis of supravalvar aortic stenosis was established by catheterization and angiocardiography and aided by EKG and 2-D echocardiography. Aortic stenosis was corrected by surgery and discharged in a good condition. A brief review of the literature was made.