Gallbladder agenesis (GA) is a rare congenital anomaly of the biliary system. Reported for the first time in human beings by Bergman back in 1702. Gallbladder agenesis (GA) is often associated with other congenital abnormalities. Patients become symptomatic in 23% of cases. GA is often misinterpreted as other diseases, therefore, leading to unnecessary surgery. We report a case of congenital GA associated to cystic duct absence without biliary tract abnormality misdiagnosed by sonography. We performed intraoperative cholangiography to exclude any biliary tract abnormality. It is present in 1/6 of cases of biliary atresia; the isolated absence of the gallbladder and cystic duct is rare.
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