Abstract: Biliary atresia is a rare congenital disease of children with unknown pathogenesis and biliary obstruction, which is mainly manifested with progressive inflammation and fibrous obstruction of the biliary system inside and outside the liver, and subsequently develop into cholestatic cirrhosis and liver failure. Although biliary atresia is rare, it is the most common cause of cholestasis during the infancy period. If surgical treatment were not performed (such as Kasai operation and liver transplantation), children may die in a short period of time. Kasai operation can improve biliary drainage, whereas it fails to change the outcomes of children with biliary atresia. Most of them will still suffer from persistent liver injury due to cholestasis, and eventually require liver transplantation. At present, there is no consensus on whether Kasai operation should be performed prior to liver transplantation. In this article, research progress on the diagnosis and surgical treatment of biliary atresia was reviewed, aiming to provide reference for clinical diagnosis and treatment of biliary atresia and improve the survival of children with biliary atresia.