Cutaneous neuroendocrine carcinoma (CNEC) is a rare tumour. We report the case of a 75-year old woman affected with a rapidly progressive CNEC of the right cheek which kept recurring despite surgical excision followed by radiotherapy. The clinical features of CNEC are not specific. The tumour is located in the dermis, and the tumoral cells (Merkel cells) have a monotonous appearance. Gould's classification of these carcinomas into three types has a prognostic value. Immunohistochemistry is a mandatory complement to light microscope examination. It is very difficult to distinguish between CNEC and metastasis from a visceral small cell carcinoma. Treatment consists of surgery combined with radiotherapy. Chemotherapy was very successful in our patient and should be considered in other cases.