The treatment of congenital diaphragmatic hernia (CDH) still represents a challenge, even for the specialised multidisciplinary teams in centres that provide treatment for CDH. Despite significant progress in the fields of pathophysiology, prenatal diagnosis, surgical techniques and intensive care, CDH is a disease still burdened with a high mortality. Due to the paucity of randomised studies, there are no standard guidelines for treatment. The present review looks at existing diagnostic and therapeutic principles based on the available literature.