Biliary atresia (BA) is the main cause of obstructive jaundice in the newborn, and it’s defined as an obliterative disorder of the intra and extrahepatic biliary tree dependent on an inflammatory-destructive process of unknown etiology. Research looking into the metabolomics profile of infants with biliary atresia has identified a different profile as compared to other children with neonatal cholestasis. This is not currently in clinical practice but may be a useful investigation in the future. Biliary atresia is the most common cause of pediatric end-stage liver disease and the leading indication for pediatric liver transplantation. Affected infants exhibit evidence of biliary obstruction within the first few weeks after birth. Early diagnosis and successful surgical drainage of bile are associated with greater survival with the child’s native liver. Unfortunately, because noncholestatic jaundice is extremely common in early infancy, it is difficult to identify the rare infant with cholestatic jaundice who has biliary atresia. Hence, the need for timely diagnosis of this disease warrants a discussion of the feasibility of screening for biliary atresia to improve outcomes. Published analyses indicate that newborn screening for biliary atresia by using serum bilirubin concentrations or stool color cards is potentially life-saving and cost effective. Further studies are necessary to evaluate the feasibility, effectiveness, and costs of potential screening strategies for early identification of biliary atresia