While most long-term survivors of biliary atresia lead lives of good quality, some patients experience late complications. Cholangitis is a common complication even among 20-year survivors including some who develop ongoing liver dysfunction. Portal hypertension is another late complication. Endoscopic treatment for esophageal varices and partial splenic embolization for hypersplenism is effective as long as hepatic functional reserve is preserved. On the other hand, the treatment of secondary intrapulmonary vascular disorders including hepatopulmonary syndrome and portopulmonary hypertension is difficult. Early liver transplantation is recommended for these conditions. Pregnancy and delivery can be stressful events potentially leading to liver failure in female patients. Even very long-term survivors should be carefully monitored in terms of liver function, portal hypertension, and cholangitis.