The Authors report a case of Merkel cell carcinoma of the cervicofacial district, particular due to its clinical and evolutive characteristics. The Merkel cell carcinoma was first identified by Toker in 1972 and is also known as a primary neuroendocrine the or trabecular carcinoma of the skin. The Merkel cell carcinoma is a rare aggressive skin cancer most frequently localized in the cervicofacial district. Although in earlier reports on the clinical history of these tumors primary emphasis was placed on their local aggressive behaviour, it has recently become evident that these neoplasms have a marked propensity for early dissemination. Distant metastases are often responsible for disease-related mortality. Merkel cell carcinoma have classically been managed with surgery alone or surgery followed by radiation therapy. Treatment of Merkel cell carcinoma with radiation therapy alone has usually been reserved for advanced unresectable tumors.