First descriptions of EHBA date from the 19th century. More than 100 years later, we are not able to decide if biliary atresia is a congenital malformation or the final stage of a peripartal infection. Epidemiological and genetical research seem to be as important as basic reseach, e.g. in animal models. Herein, the close cooperation of clinically and scientifically orientated disciplines is inevitable.