The most serious complications of liver hydatid cyst disease are fistulization into biliary tract, compression of adjacent vascular structures, anaphylactic reaction, and perforation. Fistulization between liver hydatid cyst and gallbladder tract is an extremely rare complication with only a few cases reported so far. Herein, we aimed to report a 43-year-old man who was diagnosed as having a cholecysto-hydatid cyst fistula. The patient presented to emergency department with signs and symptoms of cholangitis. His biochemical tests revealed elevated AST, ALT, GGT, and bilirubin levels. The radiological examinations (CT, MRCP) revealed a lesion consistent with hydatid cyst (Hydatid cyst ELISA IgG +) with an approximate size of 90*65 mm, which was posterolateral to the gallbladder and fistulized into the latter. In order to relieve pressure within the biliary tract, ERCP with sphincterotomy was performed. He was taken to the operating room a few days later. After draping sponges soaked with 3% NaCl onto the surgical field, near-total pericystectomy + omentopexy + cholecystectomy + common bile duct exploration + T-tube drainage were performed. Bile duct opening to the posterior wall of the cyst was sutured with a prolene suture. Albendazole treatment was started on first postoperative day. After taking a cholangiogram on 21st postoperative day, the T-tube was removed without any complication. In conclusion, cholecysto-hydatid cyst fistula is an extremely rare complication of hydatid cyst disease even in endemic regions. The gold standard for the diagnosis is the combined use of characteristics of clinical presentation, biochemical parameters, and radiological studies. Treatment plan is designed on the basis of the relationship of a fistulized cyst with other bile ducts. KEY WORD: Cholecysto-Hydatid Cyst Fistula, Gallbladder, Hydatid Cyst, Liver.

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