Biliary atresia affected infants are completely normal for the first two weeks of their life. After two weeks, they slowly develop jaundice, i.e. yellowish discoloration of the skin and sclera of the eyes. This is due to accumulation of bile in the liver. The urine passed by the infant is dark yellow colored and the stools are clay colored. Jaundice is common in the first two weeks of life and can be easily treated. But sudden appearance of jaundice or continued jaundice after two weeks of birth should alarm the parents and the doctor. The infant should then be investigated for biliary atresia.

Due to collection of bile in liver, the liver gets inflamed and swollen. This leads to abdominal bloating or distension in the infant. There is increased itching due to excess bile which makes the infant irritable. Due to continued inflammation and poor digestion, the infant progressively loses weight and appears ill and toxic.