Agenesis of the gallbladder is a rare anomaly of the biliary system. Only 200 cases have thus far been reported in the world medical references. The authors have presented a case with a congenitally absent gallbladder and cystic duct. The diagnosis was made exclusively with peroperative cholangiography, because preoperative radiologic attempts mimics an obstructed cystic duct. The incidence of congenital absence of the gallbladder and cystic duct is between 0.01 and 0.015%. The case presented here was operated on with a preoperative diagnosis of an obstructed cystic duct. The agenesis was verified during surgery using peroperative radiomanometry and cholangiography. The importance of this anomaly lies in its recognition because it can be accompanied by choledocholithiasis. In as much as choledocholithiasis is coexistant it must be treated with the standard surgical procedures.

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