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Biliary atresia is an unknown etiology of extrahepatic bile duct obstruction with a ‘fibrous ductal remnant,’ which represents the obliterated ductal remnant in the porta hepatis. The sonographic ‘triangular cord’ (TC) sign has been reported to indicate a fibrous ductal remnant in the porta hepatis. In this review, we discuss the correlations among surgicopathological and sonographic findings of the porta hepatis and the definition, objective criteria, diagnostic accuracy, and differential diagnosis of the TC sign in biliary atresia.