Biliary atresia (BA) is a progressive, idiopathic, fibro–obliterative disease of the extrahepatic biliary tree that presents with biliary obstruction exclusively in the neonatal period. It has been listed as a rare disease by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH) – the incidence is as low as only 1 patient in 10,000 to 20,000 live births. However, Biliary Atresia is the most common surgical cause of neonatal jaundice for which Liver Transplantation is indicated. We report a rare case of Biliary Atresia in a 6 months old female child who presented with jaundice and abdominal distension.