Abstract
Choledochal cysts and familial adenomatous polyposis are infrequent disorders that are often manifest in childhood or in early adult life. The rarity and early presentation of these diseases suggests a genetic basis, which has been established for familial polyposis but not for choledochal cysts. We report a case of a 26-yr-old woman with both disorders and offer an alternative genetics-based etiology for the formation of choledochal cysts.