The outcome data of 132 patients treated at the Department of Pediatric Surgery in Mainz during the last 25 years were reviewed. Prenatal diagnosis of abdominal wall defects (AWDs) and associated malformations led to increasing selection of the patient population. The aim of primary closure of the abdominal wall can be achieved more frequently in gastroschisis (GS) than omphalocele (OC), while the postoperative course is more complicated and of longer duration in GS. Delayed or secondary closure extended the hospitalization period but had no negative effects on the outcome. Reoperations or planned secondary operations were performed in 23 patients with GS and 14 with OC. Early mortality was 15/55 for OC and 21/77 for GS over the period of 25 years. In recent years, a drastic reduction in mortality has occurred, and mortality is now mainly due to additional malformations. Further development and quality of life are not significantly reduced after survival of an isolated AWD. Malpositioning of parenchymatous organs after closure of AWDs has to be considered during pregnancy and abdominal operations.