Immune checkpoint inhibition (ICI) has been acknowledged as a breakthrough treatment in multiple advanced cancer types. This is also the case in metastatic Merkel Cell Carcinoma (MCC), a disease that is historically associated with a poor prognosis. Recently, several randomized trials demonstrated superior results of ICI compared to chemotherapeutic agents in patients with metastatic MCC, with less toxicity, an increased overall survival (OS), and more durable responses. Therefore, ICI is now generally considered as a new standard treatment option in this setting.