Hypertensive encephalopathy: a rare presentation of Williams-Beuren Syndrome

A male child of four years is reported with Williams-Beuren Syndrome (WBS). It was not recognized initially when he presented with odd facies and developmental delay since early infancy. The diagnosis was established later when he developed hypertensive encephalopathy secondary to bilateral renal artery stenosis, a congenital anomaly that must be looked for in such patients. No echographic evidence of congenital heart disease was found. Blood pressure estimation on routine physical examination of every child is emphasized. The diagnosis is mainly clinical as the definitive chromosomal studies are presently not available in Pakistan.