Agenesis of the gallbladder – the complete absence of a gallbladder, is a very rare anomaly. The low occurrence of agenesis of the gallbladder is confirmed by pathoanatomic studies, the frequency, according to autopsy data, is 0.03- 0.09%. Gallbladder agenesis manifests itself in different ways: in 50% of patients, symptoms mimic biliary colic, and in 35% they are detected accidentally during surgery or autopsy, and another 15% may have fatal fetal abnormalities. It was noted that in most cases agenesis was combined with other malformations of the biliary tract; in women, the anomaly occurs 2 times more often. A rare clinical case of gallbladder agenesis diagnosed during surgery from our practice is presented.