Biliary atresia (BA) is a rare, devastating disease of infancy where a fibroinflammatory process destroys the bile ducts. BA is the most common indication for childhood liver transplantation (LT). BA treatment is started with a portoenterostomy (PE) operation and adjuvant medical therapy, and continued with LT if the PE fails. The aim of this study was to investigate the incidence of BA in Finland and to evaluate the outcomes of Finnish BA patients in the era of liver transplantation, with special emphasis on the effects of treatment centralization in 2005. The occurrence and predictors for esophageal varices, the relations between liver histology and clinical outcome variables evaluated, and noninvasive follow-up tools identified. BA patients born in Finland between 1987 and 2010 were identified from the national Register of Congenital Malformations and Children s Hospital database. All hospital records were reviewed for diagnosis confirmation, associated anomalies, treatment, follow-up data, LT, and outcome. Liver biopsies taken at PE and LT were reviewed together with follow-up biopsies. BA was diagnosed in 74 children. The incidence of BA was 1:19 900 live births. Anomalies associated with laterality disorders were observed in 17 (23%) patients. Births with BA were more common in autumn-winter than in spring-summer (p=0.013). After centralization, the clearance of jaundice rate improved significantly from 29% to 73%, p=0.001. This improvement translated into increased native liver survival: before centralization the native liver survival at four years was 21% and after centralization 73% (95% confidence interval, CI 54-91%), p