Between September 1955 and January 1990, 94 pediatric patients were managed for renovascular hypertension caused by renal artery occlusive disease. Patients (50 boys and 44 girls) were aged 4 days to 17 years (median age: 7 years). At initial evaluation, 34 patients had symptoms of hypertensive encephalopathy or acute heart failure, 36 had moderate symptoms, and 24 were symptom-free. Sixty-five cases were classified on the basis of clinical, radiological, and histological features, as follows: neurofibromatosis (17), fibromuscular dysplasia (11), diffuse arterial calcified elastopathy (11), renal artery thrombosis (10), Williams syndrome (4), Takayashu disease (3), and miscellaneous diseases (9). In the 29 remaining patients, classification was based only on radiological features: causes included unilateral renal artery stenosis (15), bilateral renal artery stenosis with or without aortic stenosis (11), and miscellaneous disorders (3). Surgical treatment consisted in 47 renal revascularization, procedures (14 aortorenal bypasses, 8 aortorenal reimplantations, 9 anastomoses in the upper mesenteric arterial system, 7 autotransplantations, 4 resection-reanastomosis procedures, and 5 miscellaneous procedures). Renal revascularization failed in 15 cases (32%) (because of thrombosis in 14 cases and dehiscence in one). Residual or recurrent stenosis was seen in 7 arteries, whereas the anatomic result was satisfactory in 25 arteries (53%). Blood pressures returned to normal in 16 of 40 (40%) patients successfully treated by revascularization. An additional 25 patients recovered normal blood pressure values after primary nephrectomy (21), partial nephrectomy (4) or nephrectomy after failed renal revascularization.(ABSTRACT TRUNCATED AT 250 WORDS)