Between 1953 and 1995, 300 patients with biliary atresia underwent surgery at Tohoku University Hospital. The 10-year survival of patients who were operated on in or before 1965 was 9%. But the survival rate went up to 61% in patients operated on between 1976 and 1985. Eighty-five patients including 2 who developed liver failure after Kasai operation and underwent liver transplantation have survived more than 10 years. Eleven of them (13%) have recurrent or persistent jaundice. Of the 30 patients who have survived more than 20 years (10 males and 20 females, age range; 20 to 41 years), 20 underwent hepatic portoenterostomy, 8 underwent hepaticoenterostomy and the remaining 2 underwent hepatic portocholecystostomy. None of these patients has undergone liver transplantation. Twenty-two patients have led near-normal lives. The remaining 8 patients have experienced some troubles due to cholangitis, portal hypertension, intrahepatic gallstones and so on. Two of them are considered as candidates for liver transplantation. While the majority of long-term survivors of biliary atresia have good quality of life, close long-term follow-up is essential even in patients with biliary atresia aged 20 years or more.