The incidence of congenital diaphragmatic hernia (CDH) may be as high as 1 in 2000. Over the past two decades, antenatal diagnosis rates have increased, the pathophysiology of CDH has become better understood, and advances in clinical care, including foetal surgery, have occurred. However, there remains a paucity of randomised controlled trials to provide evidence-based management guidelines. Reports of improved survival rates appear to be confined to a select subset of CDH infants, surviving to surgical repair, while the overall mortality, at over 60%, appears to be unchanged, largely due to the often forgotten ‘hidden mortality’ of CDH. The significant long-term morbidity in surviving infants has become apparent, and the need for long-term multidisciplinary follow up established. A total of 10% of cases may present later in life, and misdiagnosis on initial chest X-ray may lead to significant morbidity.