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Gallbladder cancer is the fifth most frequent women cancer in Algeria. Squamous-cell and adenosquamous carcinomas are the rarest histological type of this cancer and represent less than 7%. Malformation of the bile ducts such as a choledochal cyst or a biliopancreatic maljunction are risk factors for this cancer. This manuscript reports a rare case of an 82-year-old woman operated on for an adenosquamous carcinoma of the gallbladder associated with a bile duct cyst and a biliopancreatic junction abnormality. Aggressive surgery was performed combined with adjuvant oral chemotherapy. Twenty-nine months of recurrence-free survival was achieved by this patient suffering from this aggressive and rare form of gallbladder cancer.