| Summary: | Choledochal cysts are cystic dilation of extrahepatic duct, intrahepatic duct, or both that may result in significant morbidity and mortality, unless identified early and managed appropriately. The incidence is common in Asian population compared with western counterpart with more than two third of the cases in Asia being reported from Japan. The traditional anatomic classification system is under debate with more focus on etiopathogenesis and other aspects of choledochal cysts. Even though categorized under the same roof, choledochal cysts vary with respect to their natural course, complications, and management. In this review, with the available literature on choledochal cysts, we discuss different views about the etiopathogenesis along with the natural course, complications, diagnosis, and surgical approach for choledochal cysts, which also explains why the traditional classification is questioned by some authors.
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