Supravalvular aortic stenosis is the left ventricle outflow tract stenosis that has the lowest rate of incidence. The stenosis frequently starts at the sinotubular area. The possibility for concordance of supravalvular aortic stenosis with Williams syndrome is high. The majority of cases develop due to a mutation in the elastin gene located in the seventh chromosome. It occurs in a large percentage of the patients as a focal stenosis shaped as an hourglass. Due to this stenosis, hypertrophy and dilatation occur in the left ventricle. It is very important to perform the surgical treatment without deteriorating the left ventricle functions and compromising the coronary arteries in patients where an intervention is indicated. This article presents an 11-year-old case who had serious supravalvular aortic stenosis and was not identified as suffering from Williams syndrome with genetic evaluation, and we discuss this rare case in the light of the latest literature data

Keywords: Aortic stenosis, Supravalvular, Child