A small-cell carcinoma of the gallbladder is an uncommon cancer with high malignancy potential. It is usually found in elderly women and is associated with cholelithiasis. These tumors show an aggressive clinical camse and death usuaUy ocans within a few months after diagnosis. The diagnosis of a small-cell carcinoma should be based on cell morphology, histologic pattern, and immunohistochemical study. Correct diagnosis is important not only because these tumors give rise to endocrine syndrome but also because the chemotherapeutic approach to a small-cell carcinoma differs from that for an adenocarcinoma. We experienced a case of a small-cell carcinoma of the gallbladder in a 73-year-old woman with acute cholecystitis syndrome. She was treatment with a palliative cholecystectomy and chemotherapy with short-term excellent response. The literature on the subject is reviewed.