The authors report a case of biliary atresia (BA) with duplication of the common bile duct. A 1-month-old girl was referred for investigation of persistent jaundice. BA was suspected and confirmed with intraoperative cholangiography. A portoenterostomy was performed at 50 days of age. During dissection of the fibrous remnant of the common bile duct, a separate fibrous bile duct remnant running in parallel on the left, was identified. Histologically, there was bile duct proliferation at the porta hepatis of the left bile duct remnant. A diagnosis of BA with duplicated common bile duct was made. Postoperative course was uneventful, and at a follow-up assessment at 7 months, she was jaundice free, and her progress has been unremarkable.