Conclusion

Williams–Beuren syndrome is a multisystem condition distinguished by common, often unacknowledged arterial hypertension, which may signify the earliest manifestation of an underlying, potentially curable arteriopathy. This case highlights a hypertensive emergency caused by severe bilateral renal artery stenosis in a child with Williams–Beuren syndrome, successfully treated with percutaneous transluminal angioplasty, resulting in sustained blood pressure control. It illustrates the necessity of checking the blood pressure of all children with WBS, starting at the moment of diagnosis and continuing during follow-up, even if there are no visible cardiac bruits or symptoms. Due to the increased prevalence and potential consequences of vascular lesions in WBS, a minimum standard for vascular imaging is crucial in any hypertensive patient, especially when hypertension is severe, refractory, or accompanied by related clinical complaints. Early detection of renovascular involvement and prompt multidisciplinary management by pediatric nephrologists, cardiologists, radiologists, and surgeons are crucial to prevent irreparable cardiovascular and renal damage and to improve long-term outcomes in these children.