Merkel cell carcinomas (also known as trabecular carcinomas) are primary cutaneous small cell neuroendocrine neoplasms with the potential to metastasize. Control of disseminated disease is therefore important. A case of metastatic Merkel cell carcinoma with an excellent response to chemotherapy is presented. The regimen chosen for this case is similar to that used for bronchogenic small cell anaplastic carcinoma. The reason for selecting this regimen was the common neuroendocrine differentiation and the similar histopathologic features of these two tumors. Only a few reports have described chemotherapy for Merkel cell carcinoma and similar agents were used. These cases are reviewed and critically analyzed.