Renovascular disease is an uncommon but important cause of hypertension in children. It is usually diagnosed after a long delay because blood pressure is infrequently measured in children and high values are generally dismissed as inaccurate. Many children with renovascular disease have abnormalities of other blood vessels (aorta, cerebral, intestinal, or iliac). Individuals suspected of having the disorder can be investigated further with CT, MRI, or renal scintigraphy done before and after administration of an angiotensin-converting-enzyme inhibitor, but angiography is still the gold standard. Most children with renovascular disease will need interventional or surgical treatment. Endovascular treatment with or without stenting will cure or reduce high blood pressure in more than half of all affected children. Surgical intervention, if needed, should be delayed preferably until an age when the child is fully grown. Modern treatment provided by a multidisciplinary team of paediatric nephrologists, interventional radiologists, and vascular surgeons offers good long-term treatment results.