Biliary atresia is a perinatal progressive disease with partial or total biliary fibrosis obstruction, a common cause of neonatal cholestasis, not an uncommon disease, with an incidence of about 1:10,000-15,000, and a higher incidence in China and Japan than in Europe and the United States. The incidence is slightly higher in female babies than in male babies, and it is generally believed that there are no racial differences, no relevant genetic factors have been found, and about 10% of cases are combined with other malformations. If left untreated, the disease will inevitably progress to cirrhosis, liver failure and death, with most surviving for less than 1 year. Progressive obstructive jaundice is the most prominent manifestation of biliary atresia, and yellowing of the baby’s skin or eyes is the earliest and easiest sign for parents to detect. Because children with biliary atresia generally have no obvious symptoms at birth, and jaundice is not a specific manifestation of biliary atresia, and overlap with physiological jaundice, which can easily cause delayed diagnosis, and some children with jaundice are not obvious, and when the symptoms are typical, the diagnosis is often delayed and the best time for early and effective treatment is missed. Therefore, when the baby is born within 1 to 2 weeks or a month, jaundice appears, and also after the physiological jaundice has subsided for 1 to 2 weeks, it appears again with progressive deepening. As the jaundice worsens, the sclera and skin change from golden yellow to greenish-brown or dark green, and the stool changes from normal yellow to white clay, the baby should be seen early at a children’s specialist hospital for early detection and diagnosis. Surgical treatment is the only effective method. Surgery is recommended before irreversible liver damage occurs, mostly within the first three months of life. The prognosis is extremely poor if biliary cirrhosis has occurred too late for surgery. Therefore, when the baby is still diagnosed with “biliary atresia” after a series of tests such as blood and liver function, ultrasound, isotope and MRI, early surgery should be performed. Intraoperative exploration and cholangiography are the gold standard for biliary atresia. The Kasai procedure is a hepatoportal-jejunostomy. The method is to take an oblique or transverse incision in the right upper abdomen under the ribs, explore the gallbladder and hepatoportal area first, and perform choledochogram during the operation to clarify whether it is biliary atresia. After the diagnosis is confirmed, the atretic extrahepatic biliary system is resected, the fibrous mass is freed upward to the fibrous mass in front of the bifurcation of the left and right branches of the portal vein, the fine branches at the upper edge of the portal vein bifurcation are ligated, and then the left and right bifurcations are pulled downward to fully expose and resect the fibrous mass of the hilar; the depth of resection of the fibrous mass of the hilar is the key to Kasai’s surgery, and generally after resection of the fibrous mass of the hilar, a very thin envelope is retained on the surface of the liver, and finally the jejunum and After resection of the fibrous mass of the hilar, a very thin layer of peritoneum should be retained on the surface of the liver, and finally the jejunum should be anastomosed with the hilar by Roux-en-Y. 2. Liver transplantation Biliary atresia is the main indication for liver transplantation in children. It is suitable for children with complete atresia of the intrahepatic and extrahepatic bile ducts, cirrhosis and failure of Kasai surgery. Currently, the main transplantation methods are DCD split liver transplantation or pro-liver transplantation. In general, children weighing 8-20 kg are more suitable for pro-liver transplantation.