The outcome of congenital diaphragmatic hernia (CDH) differs for different stages of the fetus or infant's life (i.e., antenatal, immediate postnatal, and postoperative). Assessing combined data from nonrandomized studies is technically difficult. Following recognized methods of reviewing such trials, we aimed to review the available literature on the outcome of CDH to provide a guide to clinicians when counselling parents who have a fetus/infant with this condition. Thirty-five studies reporting data for CDH from 1985 to March 1998 were identified using a high sensitive search strategy, hand-searching journals, and reviewing references of relevant studies. These were systematically reviewed.The median overall mortality was 58% (interquartile range (IQR), 43-65%) for babies diagnosed in utero, 48% (IQR, 35-55%) if born alive, and 33% (IQR, 18-54%) postoperatively. Diagnosis before 25 weeks of gestation is not a uniformly bad prognostic indicator (median mortality, 60%). Outcome was worse for those fetuses with other anomalies (median mortality, 93%). The median percentage mortality for all infants born alive and treated in extracorporeal membrane oxygenation (ECMO) centers was 34% (IQR, 26-47%). Median percentage mortality for all ECMO-treated infants was 44% (IQR, 35-50%). Different treatment strategies may have a variable impact on outcome. These figures, together with local data, may help in parental counselling on prognosis for fetuses/infants with CDH.