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William's syndrome is a genetic disorder that is associated with a spectrum of cardiovascular disease: congenital heart disease, vascular disease, coronary artery disease, hypertension and arrhythmias. Patients may be at risk of significant morbidity and mortality over their lifetime including a risk of sudden cardiac arrest. Williams syndrome patients may develop progressive multi-site arterial stenosis, systemic hypertension, valve dysplasia, and arrhythmias with advancing age. They should always be considered at risk of having underlying coronary disease prior to anesthesia. Standard screening includes a comprehensive physical exam, electrocardiogram, and echocardiogram; however advanced cardiac imaging with computed tomography and/or magnetic resonance angiography may be beneficial to fully assess the vascular tree. We will review a systematic approach for clinicians caring for Williams syndrome patients to use for the detection of overt and subclinical forms of cardiovascular disease, especially prior to procedures requiring general anesthesia. • Williams syndrome comprehensive cardiovascular screening • Advanced cardiac imaging is beneficial • Williams syndrome patients who are undergoing anesthesia should be assumed to have risk of coronary disease. [ABSTRACT FROM AUTHOR]