Merkel cell carcinoma (MCC) is a highly aggressive neuroendocrine carcinoma of the skin demonstrating a high rate of recurrence and metastasis. Indeed, 5-year rates for MCC specific survival are only about 60%. Although MCCs’ incidence is rapidly increasing, it is still a very rare tumour. In this regard, the American Cancer Society had estimated for 2008 almost 1500 new cases in the USA. Recently, the newly identified Merkel cell polyomavirus has been found associated with most of the MCC cases. Nevertheless, the pathogenesis of MCC is not yet fully understood. Here, we will summarize recent findings of the pathogenesis of MCC, present an overview of clinical aspects and discuss treatment options for MCCs.