This report describes the first in utero repair of diaphragmatic hernia in Australia. The patient was a 32 year old woman with major infertility problems who was diagnosed at 15 weeks gestation as having an infant with diaphragmatic hernia. After extensive consideration and counselling the parents requested in utero repair. This was performed at 28 weeks gestation and was technically successful, but the infant was found to be dead after uterine closure. The mother has subsequently been delivered of normal twins at term by Caesarean section. In utero repair of diaphragmatic hernia requires a high degree of team work, is technically demanding and has major ethical implications. It should be restricted to nationally designated units.