In a large paediatric renal unit over the last 14 years, 19 children (10 male and 9 female, aged 1 week to 16 years, mean 7 years) with renal artery stenosis (RAS) were evaluated. Transplant RAS cases were not included. All 19 children were hypertensive. In 10 this was an incidental finding. Based on clinical findings and arteriography, the causes of RAS included a middle aortic syndrome (MAS) (n = 5), neurofibromatosis (n = 3), William's syndrome (n = 3), fibromuscular hyperplasia (FMH) (n = 4), idiopathic RAS (n = 2) and isolated branch artery stenosis (n = 2). Previous studies have suggested FMH is the commonest cause of RAS in the paediatric population. In our study the largest subgroup are MAS/William's syndrome children, in whom the angiographic appearances were indistinguishable. Where possible, management, both surgical and radiological, and eventual outcomes have been described.