Merkel cell carcinoma (MCC) is a rare, aggressive neuroendocrine skin cancer localized mostly on exposed, sun-damaged skin – on the head and neck. Although rapidly increasing incidence of MCC is observed, the pathogenesis of the tumour is unclear. Diagnosis is based on clinical and histopathological examination together with immunohistochemistry. Discovery of the Merkel cell polyomavirus and its association with Merkel cell carcinoma may significantly contribute to understanding of MCC pathogenesis and development of new methods of therapy. Surgery with adjuvant radiotherapy is the essential treatment of the localized disease.