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We report a patient with two synchronous foci of small cell carcinoma in the rectosigmoid region, metastatic to the liver and paraaortic lymph nodes, and review forty-five other reported cases. Most frequent in the rectosigmoid and cecal regions, these tumors are highly aggressive, and distant metastasis at presentation is almost always the rule. The mean survival is approximately 6 months, and 1 year survival only 15%. A tentative management strategy is proposed.