Merkel cell carcinoma is a rare but aggressive neuroendocrine carcinoma of the skin with a rising incidence and a high mortality rate. It occurs primarily in sun-exposed skin of older individuals. It is characterized by a high rate of local recurrence, regional lymph node metastases and distant metastases, occurring even after prompt treatment. Many controversies exist regarding its pathogenesis and optimal management. The discovery of Merkel cell polyomavirus has been a major breakthrough in understanding the aetiology of the disease. A recently adopted new international consensus staging system in combination with new international diagnostic codes are expected to facilitate future clinical trials and improve the management of patients. According to recent (2010) guidelines, most patients should be managed with a combination of surgery and radiotherapy.