A case of agenesis of the gall bladder and cystic duct with associated choledocholithiasis and suppurative cholangitis has been presented. Incidence of the congenital disorder, its symptomatology and associated pathology have been reviewed. Mechanisms responsible for common duct dilatation in the absence of calculi as well as for the formation of calculi in the absence of the gall bladder are discussed. A rational therapeutic approach is outlined.
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