Due to better methods of diagnosis, an increasing number of children with renovascular hypertension are being discovered. The disease itself has variable clinical manifestations, and an adequate laboratorial workup, including arteriography and renin assays, is mandatory in suspected cases. Treatment depends on the prognostic values of the renin assays and surgery cures 80% of the patients, either by nephrectomy or by revascularization techniques. Among these, renal autotransplantation gives better exposure of the operative field, and therefore better results. In most cases, systemic infection seems to be the main cause of the inflammatory reaction within the arterial wall that causes obstruction.