OBJECTIVES: This study analyses the prognosis of Biliary Atresia (BA) in France since 1986, when both Kasai operation (KOp) and Liver Transplantation (LT) became widely available. METHODS: The charts of all patients diagnosed with BA born between 1986 and 2015 and living in France were reviewed. RESULTS: 1428 patients were included; 1340 (94%) underwent KOp. Total clearance of jaundice (total bilirubin </=20 mumol/l) was documented in 516 patients (39%). Age at KOp (median 59 days, range 6-199) was stable over time. Survival with Native Liver (SNL) after KOp was 41%, 35%, 26% and 22% at 5, 10, 20 and 30 years, stable in the 4 cohorts. 25-year SNL was 38%, 27%, 22%, 19% in patients operated in the 1, 2, 3 month of life or later, respectively (p = 0.0001). Center caseloads had a significant impact on results in the 1986-96 cohort only.16%, 7%, 7%, 8% of patients died without LT in the 4 cohorts (p = 0.0001).753 patients (55%) underwent LT. Patient survival after LT was 79% at 28 years. 5-year patient survival after LT was 76%, 91%, 88%, and 92% in cohorts 1 to 4, respectively (p < 0.0001),Actual BA patient survival (from diagnosis) was 81%. 5-year BA patient survival was 72%, 88%, 87%, 87% in cohorts 1986-96, 1997-2002, 2003-09, 2010-15, respectively (p < 0.0001). CONCLUSIONS: In France, 87% of BA patients survive nowadays and 22% reach the age of 30 years without transplantation. Improvement of BA prognosis is mainly due to reduced mortality before LT and better outcomes after LT.< Réduire