Biliary Atresia (BA) affects approximately 1:20,000 babies and is the most frequent cause of surgical jaundice in children. BA presents in the first few weeks of life with pale stools and dark urine in otherwise healthy infants. It is the end result of a destructive inflammatory process of the bile ducts, with unclear origins. BA is a condition seemingly unique to the neonatal period, characterized by obliteration of both intra and extrahepatic bile ducts and amenable to surgical treatment by Kasai portoenterostomy, in the attempt to restore bile flow. In the most experienced centers, approximately half of children who underwent Kasai portoenterostomy achieve jaundice clearance, avoiding a rapid referral to liver transplantation, required in those in whom this approach is unsuccessful.