Cystic biliary atresia: A distinct clinical entity that may mimic choledochal cyst

Cystic biliary atresia (CBA) is a relatively uncommon but clinically significant variant of biliary atresia. The presence of a cyst in the hepatic hilum on imaging in an infant with cholestasis supports the diagnosis of CBA, but can also be seen in patients with a choledochal cyst—the main differential diagnosis in patients with CBA. The reported case outlines the clinical presentation and imaging findings in a patient with surgically confirmed and treated CBA and emphasizes the importance of distinguishing CBA from choledochal cyst at diagnostic imaging given the disparate timing and type of surgical treatment necessary for successful management of these distinct entities.