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Background: Gallbladder agenesis is a biliary tract related congenital malformation with an incidence of 10-65 per 100,000 and associated with other congenital abnormalities. GA is usually asymptomatic, but sometimes patients become symptomatic. The most usual symptoms are jaundice, abdominal pain in the right upper quadrant, nausea and vomiting. We reported a case of GA and choledochogastric fistula in a patient with history of cholangitis. Case Presentation: A 70-year-old man presented to Emergency Department of Modarres Hospital with jaundice, fever, right upper quadrant abdominal pain, nausea and vomiting. Clinical examination and lab test demonstrated cholangitis. He underwent ultrasonography, abdominopelvic CT scan and ERCP. ERCP revealed a CBD fistula. Due to recurrent symptoms, he underwent operation and hepaticojejunostomy was done. Conclusion: In our knowledge, the case of GA and choledochogastric fistula is rare. Furthermore, this type of abnormalities rarely presented with cholangitis, so probable anatomical malformation of the biliary tract should always be considered as a differential diagnosis in patients with biliary disease signs and symptoms. [ABSTRACT FROM AUTHOR]