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Biliary atresia is a rare, neonatal, progressive cholestatic liver disease caused by fibroinflammatory obstruction of the biliary tree. Typical symptoms include prolonged neonatal jaundice, pale stools, and conjugated hyperbilirubinemia. Treatment starts with portoenterostomy, where fibrotic remnants of the extra hepatic bile ducts are replaced with small intestine. Best operative results are achieved among the youngest patients treated in specialized centres. Despite clearance of jaundice after a successful operation, fibrotic change of the liver continues in most. Liver transplantation serves as a salvage procedure if the portoenterostomy fails, or complications of liver cirrhosis develop after an initially successful portoenterostomy. In Finland, biliary atresia treatment was centralized in 2005. Of the patients treated thereafter, 90% are alive and over 80% with their native livers.