Hyponatremic hypertensive syndrome (HHS) is an uncommon disorder, in which hypertension is associated with profound hyponatremia. It is mostly encountered in adults with unilateral renal artery stenosis. Although renovascular hypertension is one of the commonest causes for severe hypertension in children, HHS is rarely reported in childhood. The authors report a 9-y old boy with hypertensive emergency and severe hyponatremia due to unilateral renal artery stenosis who was successfully managed by vascular stenting of the affected vessel. Prompt recognition of this disorder can be life-saving and can subsequently lead to appropriate referral and treatment as in the present case.