Agenesis of the gallbladder and cystic duct is a rare congenital anomaly occurring in <0.1% of the population. However, combined gallbladder and cystic duct agenesis (CDA) with polycystic liver disease associated with recurrent acute pancreatitis (RAP) has not been reported earlier. Herein we report a case of a 36-year-old female patient who was admitted to the hospital and successfully treated for acute pancreatitis most probably caused in the background of gallbladder and CDA with polycystic liver disease. In case of non-visualization of gallbladder with the presence of biliary symptoms after repeated ultrasonographic examinations, advanced techniques like MRCP, computed tomography, EUS and even endoscopic retrograde cholangiopancreatography (ERCP) to visualize biliary anatomy must be conducted before any surgical intervention. We present a case of gallbladder and CDA causing RAP by the formation of microlithiasis treated successfully with ERCP and without any unnecessary surgery, its management and review of the literature is assessed.
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