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Gallbladder agenesis (GA) is a rare congenital anomaly of the biliary system often associated with other congenital abnormalities. Patients become symptomatic in 23% of cases (Richards et al., 1993). GA is often misinterpreted as other diseases, therefore, leading to unnecessary surgery. Many of these patients develop a typical symptomatology of cholelithiasis that leads them to operating theatre. If an operative procedure is done, it is better to remain at the level of laparoscopy because further surgical investigation may lead to detrimental biliary tract injuries (Waisberg et al., 2002). We present a case of GA, diagnosed by magnetic resonance with cholangiopancreatography.