The clinical features of 10 cases with the adjunct of a literature review of primary neuroendocrine carcinoma of the skin (Merkel cell tumor) are reported. This cancer arises in the dermis and subcutaneous tissue of elderly individuals. Natural history is characterized by local recurrences (40%), regional lymph-nodes metastases (50%), and distant metastases (60%). Surgery is the elective treatment of primary and locoregional disease and subsequent radiotherapy prevents local recurrences and prolong disease-free survival in literature reports. Chemotherapy and radiotherary resulted in only a short-term palliative response in the metastatic setting.