Congenital diaphragmatic hernia is an uncommon congenital anomaly of the diaphragm with pulmonary hypoplasia and persistent pulmonary hypertension as serious consequences. Despite recent advances in therapy, congenital diaphragmatic hernia remains a challenging condition. Best treatment strategies are still largely unknown, and practice strategies vary widely among different centres. Additionally, as congenital diaphragmatic hernia is a relatively uncommon condition, it is difficult to recruit sufficient numbers of patients for clinical trials. In recent years, survival rates of congenital diaphragmatic hernia patients appear to have increased. With the progressively improved survival rates, the long-term prognosis and quality of life of patients have become an increasingly important issue. Survivors have been shown to be at risk for many long-term morbidities, which highlights the importance of long-term follow-up of these children. The aim of this review is to give an overview of the current knowledge regarding congenital diaphragmatic hernia.