Merkel cell carcinoma is a rare form of skin cancer of likely neuroendocrine origin wich affects mainly white population in sun-exposed areas. It is an aggressive tumor and survival is dependent on stage at the time of diagnosis. The staging evaluation include CT imaging and recently PET scan. Surgical excision with or without lymph node dissection, followed by postoperative radiotherapy in stage II disease, is the standard treatment of non metastatic disease. The role of adjuvant chemotherapy is still controversial. In patients with metastatic disease, chemotherapy regimens active in small cell lung cancer are generally used. The combination of cyclophosphamide, doxorubicin and vincristine (CAV) has an overall response rate of 75%, whereas the response rate of etoposide in combination with cisplatin or carboplatin is 60%. Experience with other therapeutic agents, such as tumor necrosis factor, interferon and octreotide is scanty. Recently, encouraging preliminary results with targeted agents have been reported. Our experience in 14 patients, four of whom treated with chemotherapy for advanced disease, is in agreement with literature