Carcinosarcoma, which comprises less than one percent of all gall bladder neoplasms, is characterized by the presence of variable proportions of carcinomatous and sarcomatous elements. Recently, several reports have described patients suffering from carcinosarcoma of the gall bladder. However, there are no large studies regarding the clinicopathologic features, therapeutic management, and surgical outcome of this disease because the number of patients who undergo resection of gall bladder carcinosarcoma at a single institution is limited. A Medline search was performed using the keywords 'gall bladder' and 'carcinosarcoma'. Additional articles were obtained from references within the papers identified by the Medline search. Optimal adjuvant chemotherapy and/or radiotherapy protocols for carcinosarcoma of the gall bladder have not been established. Curative surgical resection offers the only chance for long-term survival from this disease. The outcome of 36 patients who underwent surgical resection for carcinosarcoma of the gall bladder was poor; the 3-year overall survival rate was only 31.0% and the median survival time was 7.0 mo. Since the postoperative prognosis of carcinosarcoma of the gall bladder is worse than that of adenocarcinoma, new adjuvant chemotherapies and/or radiation techniques are essential for improvement of surgical outcome.