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Extra‐hepatic biliary atresia occurs in approximately 1:15000 live births leading to about 50 new cases/year in the UK. Presentation is with prolonged jaundice, usually in a term baby who develops signs of obstructive jaundice. Management has been improved by public and professional education to encourage early referral and diagnosis to facilitate initial surgery before 8 weeks of age. Surgical management is complementary and includes an attempt to restore biliary flow (the Kasai portoenterostomy) and liver transplantation if necessary. Medical management consists of antibiotics, ursodeoxycholic acid to encourage bile flow, fat soluble vitamin supplementation and nutritional support. Centralising surgery to specialised centres has improved survival of this potentially fatal disease to over 90% in the UK. Over half of infants undergoing portoenterostomy will clear the jaundice and have a greater than 80% chance of a good quality of life, reaching adolescence without transplantation. For those children developing intractable complications of cirrhosis and portal hypertension, liver transplantation provides a 90% chance of achieving normal life.