Merkel cell carcinoma is a neuroendocrine skin carcinoma caused by the Merkel cell virus and ultraviolet radiation. Approximately 25 Danish patients are diagnosed each year. Merkel cell carcinoma is often located on the sun-exposed areas of the skin and definitive diagnosis is made by the pathologist. Patients are treated at the department of plastic surgery and oncology with treatment modalities including surgery, radiotherapy, immunotherapy and chemotherapy as summarised in this review.