We report two cases of biliary atresia (BA) in which living-donor liver transplantation (LDLT) was performed owing to intractable cholangitis after additional hepatic portojejunostomy. [Case 1] The patient was a 15-year-old girl. Hepatic portojejunostomy was performed to treat BA. Owing to poor bile excretion, reanastomosis was performed at 2 months of age using a backflow prevention valve. Intractable cholangitis with jaundice appeared 15 years after the operation. An ileus duct was placed for jejunal stenosis using a regurgitation prevention valve; however, the intrahepatic bile duct dilatation and jaundice did not improve, and LDLT was performed. [Case 2] The patient was a 26-year-old man. Hepatic portojejunostomy was performed using a backflow prevention valve. The patient’s jaundice worsened 25 years after the operation and he was transferred to our department; however, the cholangitis did not improve and LDLT was performed. An antireflux valve may cause cholestatic cholangitis in patients with long-term BA.