Severe respiratory distress is the major cause of perinatal mortality associated with congenital urinary tract anomalies or severe obstructive uropathy, and is often accompanied by oligohydramnios. In the past the prognosis in these high risk cases was extremely poor. We review our recent experience in 3 neonates with severe respiratory distress and congenital urinary tract anomalies who were successfully managed with extracorporeal membrane oxygenation, of whom 2 had posterior urethral valves and 1 had bilateral solid renal dysplasia. The use of extracorporeal membrane oxygenation for life threatening respiratory distress in the neonate associated with congenital uropathy is not limited to our experience. A review of the National Neonatal Extracorporeal Membrane Oxygenation Registry for neonates with urological indications for extracorporeal membrane oxygenation revealed 10 interventions with 9 survivors, for a survival rate of 90%. Extracorporeal membrane oxygenation may provide cardiopulmonary support during a period of immature circulation, allowing pulmonary parenchymal and vascular maturation as well as preventing iatrogenic barotrauma.