Renovascular disease is a frequent cause of severe hypertension in children and may result in significant morbidity or mortality. Most children presenting with renovascular hypertension have few if any symptoms, but devastating neurologic injury and congestive heart failure are still too often observed. Several new radiographic techniques have been used to detect renovascular lesions, but none has yet demonstrated consistently superior results when compared with intra-arterial digital subtraction angiography. Renal venous renin sampling, duplex ultrasonography, and captopril-enhanced renal scintigraphy may be useful diagnostic adjuncts. Therapeutic objectives include cure of hypertension and restoration or preservation of renal function. At many institutions, percutaneous transluminal angioplasty has become the treatment of choice for patients with renal transplant artery stenosis and discrete, nonostial stenoses caused by fibromuscular dysplasia. More extensive lesions generally respond well to surgical correction. Chronic pharmacologic therapy is reserved for patients who do not respond to angioplasty or in cases in which the location or extent of involvement of the renal arterial system precludes surgical revascularization. Nephrectomy is usually reserved for kidneys that have minuscule function of irreparable vascular anomalies. An individualized approach to therapy is increasingly emphasized.