Gastroschisis and omphalocele (exomphalos) are embryologically distinct, and for this reason each has its own distinct constellation of associated anomalies. The perinatal management of both gastroschisis and omphalocele addresses the controversy as to the best mode of delivery that has been expressed in the literature with regard to gastroschisis and has exercised many of those involved in antenatal diagnosis in the case of omphalocele. The post-delivery management of both of these defects is briefly discussed. Controversies surrounding the aetiology of renal dysplasia and whether urinary tract obstruction is a cause of dysplasia or an anomaly associated with dysplasia are also discussed. The options for therapy and the indications for an in utero shunting procedure are outlined as well as the controversy centred around the utility of an in utero shunt. The need for a prospective randomised trial is emphasised, together with the difficulty that is raised by the need to choose a limited number of prospective indices on which treatment decisions in such a trial must be based. The need for a long-term follow-up of all patients entered in such a trial is emphasised.