Cystic biliary atresia: why is it important to distinguish this from congenital choledochal cyst?

We present a 2-month-old jaundiced infant with a rare type of biliary atresia who appeared to have a choledochal cyst on magnetic resonance cholangiopancreatography [MRCP] and ultrasound. Intra-operative findings were the only proof of biliary atresia. Following portoenterostomy, the liver function tests [LFTs] and bilirubin levels were returned within normal range and his jaundice was resolved. Any neonate presenting to a pediatric clinic with prolonged jaundice lasting more than two weeks, especially in cases of direct hyperbilirubinemia, must be thoroughly assessed and referred as early as possible for a pediatric surgical opinion to rule out the possibility of biliary atresia