Congenital biliary atresia (CBA) is a hepatobiliary system disease caused by intrahepatic/extrahepatic biliary atresia, which is the main pathogenesis of obstructive jaundice in infants. The etiology of CBA remains unknown, which may be associated with genetic variation, virus infection and immune damage. Targeted preventive measures are lacking. At present, the core of diagnosis and treatment lies in early diagnosis and surgical intervention. However, multiple challenges remain to be resolved in clinical practice. CBA infants are manifested with persistent jaundice, or yellow discoloration of the skin and sclera recurring after physiological jaundice subsides, accompanied by dark color urine and even dark tea-colored urine. The stool color became lighter, even appearing greyish-white color. Laparoscopic or open bile duct exploration is currently considered as the "gold standard" for clinical diagnosis of CBA. In recent years, use of neonatal stool colorometric scale and novel biomarkers (such as serum matrix metalloproteinase 7) have improved the early detection rate. Kasai portoenterostomy procedure is the optimal treatment for CBA, and the ideal timing is within 45 days after birth. Baseline status, type of biliary atresia and degree of liver fibrosis of CBA infants are key factors to determine the efficacy of Kasai procedure. Long-term postoperative follow-up combined with ursodeoxycholic acid, antibiotics and nutritional support can prevent the progression of liver fibrosis.