There is still much to learn about biliary atresia (BA), why it happens and how it happens being good examples of areas where there is too much speculation and not enough hard evidence. What is only too obvious is that if it is untreated, it progresses to end-stage cirrhosis and is potentially fatal within the first 12–18 months. A treatment strategy has evolved, which, in the best hands, will give a 90% chance of long-term survival for all infants born with the disease, but even in these survivors, there are still significant morbidity and problems to overcome. Nonetheless, it is compatible with normal life.