Biliary atresia is a common pediatric surgical condition caused by obstruction of the biliary tract and impaired bile drainage, ultimately leading to liver failure and endangering the life of the child. The main manifestations are progressive jaundice, hepatosplenomegaly and light-colored stools. The etiology of the disease is still unclear, and there are various theories that it may be related to viral infections, genetic mutations and immune factors. It is currently believed that biliary atresia is not a single-factor disease, but most likely a disease with common clinical manifestations due to the simultaneous action of different etiologies. Surgical treatment is the only effective treatment for biliary atresia and includes the Gussy procedure (i.e., hepatic portal-jejunostomy) as well as various modified procedures and liver transplantation. The goal of the Gussy procedure is to restore normal biliary flow in order to achieve improved liver function and long-term survival. However, postoperative complications such as cholangitis, portal hypertension, gastrointestinal bleeding, intrahepatic bile duct dilatation, and liver failure may occur, and the long-term outcome remains unsatisfactory. To prevent complications, postoperative drug therapy is necessary. Currently, the main pharmacological treatment is postoperative application of ursodeoxycholic acid, steroid hormones and long-term prophylactic application of antibiotics, the main purpose of which is to prevent bacterial cholangitis and improve bile drainage, while improving the nutritional status of the child is also essential. If the jaundice does not subside or if biliary cirrhosis occurs after surgery, liver transplantation is the only treatment. Since liver damage caused by biliary atresia is progressive, delayed surgery results in a corresponding decrease in the therapeutic effect, aggravation of biliary cirrhosis becomes irreversible, and finally death from liver failure, early diagnosis, early surgery, and postoperative medication are three important aspects to improve the prognosis of the child.