Objective: To summarize the clinicopathologic characteristics of primary adenosquamous carcinoma of the gallbladder and its diagnosis and treatment. Methods: The clinical data of 4 patients with adenosquamous carcinoma of the gallbladder admitted from 1998 to 2012 were retrospectively analyzed. Results: Of the 4 patients, 3 cases underwent radical resection and one case underwent palliative surgery. Postoperative pathological reports showed the tumor tissues contained features of both adenocarcinoma and squamous cell carcinoma, with both CK8/18 and CK5/64 positive expressions. All the 4 patients died within one year after surgery due to tumor recurrence or metastasis, with a median survival time of 180 d. Conclusion: Adenosquamous carcinoma of the gallbladder is very rare, and characterized by high malignant potential, lack of specific clinical manifestations and often seen at advanced stages. No effective treatment is available for this condition at present, surgical treatment is still the main option, and its overall prognosis is worse than that of gallbladder adenocarcinomas.