Abstract Cystic biliary atresia is a rare (5-10%) variant of type I biliary atresia. The present report intends to share our experience with cystic variant of biliary atresia along with a review of pertinent literature. The first case was a 3-month-old infant with cholestatic jaundice with preoperative diagnosis of an infantile type of choledochal cyst. The second case was a 4-month-old twin infant with cholestatic jaundice and with preoperative differential diagnoses of either cystic biliary atresia or an infantile type of choledochal cyst (cyst located within the hepatic hilum). Cystic biliary atresia was confirmed intra-operatively in both the patients. In first infant, Kasai portoenterostomy procedure was performed, while in the second patient hepaticoduodenostomy procedure was performed. At 6 months follow-up, there was no episode of cholangitis in first and two episodes in second case. In both patients’ total bilirubin was trending down on follow-up. Cystic biliary atresia presents with cystic dilatation of proximal biliary tract. Type I cystic biliary atresia may masquerade as infantile variant of choledochal cyst. The Kasai portoenterostomy procedure is a standard palliative surgical procedure for patients with biliary atresia to restore bile drainage. A high index of suspicion with early operative intervention is recommended to palliate cholestasis in the infant with cystic variant of biliary atresia.
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