Background and Objectives: Small cell carcinoma of the gallbladder is rare with only 36 cases reported in the literature. Early reports demonstrated an extremely poor prognosis for this histologic type. Five new cases are reported and the previous experience in the literature is reviewed to further clarify the clinical behavior of this malignancy. Methods: A retrospective analysis is performed on 5 new cases. An extensive review of the literature is also performed. Results: Twenty-eight pure small cell carcinomas and 8 combined small cell carcinomas with adenocarcinoma are reported in the literature. Conclusions from the literature review reveal that small cell carcinoma of the gallbladder affects an elderly patient population (median age 65 years), has a female preponderance (76%), is associated with cholelithiasis (72%), metastasizes to nodes (88%), liver (88%), lung (23%), and peritoneum (19%), and has a median survival of 4 months. Pure tumors had median survivals of 9 months and combined tumors had median survivals of 4.5 months. The 5 patients in the literature treated with surgery and chemotherapy had improved median survival of 13 months. The 5 newly reported cases had similar epidemiological characteristics to the literature data, however, these cases were managed aggressively with surgery and chemotherapy, demonstrating a median survival of 31 months. Conclusions: Although small cell carcinoma of the gallbladder is a distinct histologic and clinical entity, it has many clinical characteristics similar to adenocarcinoma of the gallbladder including comparable natural history and tendency for locoregional spread. Aggressive multimodality therapy, especially the combination of surgery and chemotherapy, may improve survival.