Thirteen patients with regional or distant metastases from Merkel cell carcinoma were treated with combination chemotherapy. Three patients had a complete response to therapy. In two patients with nodal disease the duration of response was 10+ and 4+ years. In addition, three patients had a partial response, and six patients had minor responses. Chemotherapy using a combination of drugs that are active against small cell carcinoma of the lung is recommended in the treatment of unresectable disease in patients with Merkel cell carcinoma; it may produce prolonged responses. While tumor regressions as a result of chemotherapy were often dramatic, once the disease progressed, the course of the disease often moved rapidly downhill and proved to be fatal.