Biliary atresia (BA) remains a devastating disease of infants. It is still a disease of largely unknown etiology although many hypotheses such as an aberrant early bile duct development, perinatal viral infection, aberrant immune response, and abnormalities of bile acids have all been suggested as possibly etiologically important. Although recent studies, using the techniques of molecular biology and immunohistochemistry, have improved the understanding of some of the inflammatory elements of BA, there is a lack of understanding of how many such disparate elements interact and relate. Clinically, the management in the majority of cases should consist of a primary portoenterostomy (Kasai procedure) to try and restore bile flow and alleviate jaundice. Transplantation should be reserved for those who develop chronic liver disease and its attendant complications. Recent series would suggest that over 50% of infants in large centers will be able to clear their jaundice and therefore have a reasonable expectation of long-term survival with a good quality-of-life.