Merkel cell carcinoma is a fast-growing neuroendocrine neoplasm with a low frequency of occurrence. The diagnosis and treatment should be managed by an experienced multidisciplinary team. The disease at a low stage may be curable. Radical treatment includes surgery with possible radiotherapy and, less frequently, perioperative chemotherapy. The risk of recurrence is high. In unresectable or metastatic disease, the standard of care was previously chemotherapy, as the tumor cells are very sensitive to cytotoxic drugs. The main problem is a high rate of secondary resistance to chemotherapy. Based on the pivotal clinical trials, the new standard of care in advanced and metastatic disease is immunotherapy (avelumab and pembrolizumab). Avelumab is the first immune check point inhibitor approved for metastatic Merkel cell carcinoma in the USA and Europe. This medicine allows for long-term response in above 30% of patients. The predictive factors of response are unknown. Pembrolizumab has been approved only in the USA for advanced and metastatic disease. There are currently ongoing clinical trials to further improve the treatment outcomes in the advanced and metastatic settings, including studies to overcome the resistance to checkpoint inhibitors, as well as trials with immunotherapy in a preoperative setting to reduce the risk of disease recurrence.