Williams syndrome is a congenital multisystem disease. Cardiovascular abnormality caused by elastin deficiency is the main cause of morbidity and mortality in Williams syndrome patients. Recent studies have found that 80% of Williams syndrome patients have cardiovascular abnormalities, most of which are arterial stenosis, especially the aortic valve stenosis and pulmonary artery stenosis. Operation is the main method to treat the stenosis of the artery, and the results of the operation on the aortic valve stenosis in most centers are good, but the effect of transcatheter intervention is still not obvious, pulmonary artery reconstruction has a good effect on the treatment of peripheral pulmonary artery stenosis. Advances in genetic diagnosis, surgical techniques and treatment regimens are expected to significantly improve cardiovascular outcomes in these patients. This article reviews the latest research progress of Williams syndrome combined with cardiovascular disease.