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Simple Summary: In this review, we discuss a rare skin cancer that occurs mostly in elderly people called "Merkel cell carcinoma" (MCC). The incidence is increasing due to ageing of the population, increased sun exposure, and the use of medication that inhibits the immune system. Unlike most other skin cancers, MCC grows rapidly and forms metastases easily. We discuss the biology and treatment of MCC. Management should be by an experienced and multidisciplinary team, and treatment must start quickly. The standard practice of MCC treatment is surgery followed by radiotherapy. However, because it concerns an elderly and often frail population, (extensive) surgery may not always be feasible due to the associated morbidity. In those situations, radiotherapy alone is a good alternative. An important new development is immunotherapy that can cause long-lasting responses in a significant proportion of the patients with recurrent or metastatic MCC. Merkel cell carcinoma (MCC) is a rare neuroendocrine tumor of the skin mainly seen in the elderly. Its incidence is rising due to ageing of the population, increased sun exposure, and the use of immunosuppressive medication. Additionally, with the availability of specific immunohistochemical markers, MCC is easier to recognize. Typically, these tumors are rapidly progressive and behave aggressively, emphasizing the need for early detection and prompt diagnostic work-up and start of treatment. In this review, the tumor biology and immunology, current diagnostic and treatment modalities, as well as new and combined therapies for MCC, are discussed. MCC is a very immunogenic tumor which offers good prospects for immunotherapy. Given its rarity, the aggressiveness, and the frail patient population it concerns, MCC should be managed in close collaboration with an experienced multidisciplinary team. [ABSTRACT FROM AUTHOR]