Merkel cell carcinoma (MCC) is a highly aggressive neuroendocrine tumor of the skin. Even if it is quite rare, the incidence has increased about two fold during the last twenty years. Mortality is higher than in malignant melanoma. Risk factors are chronic UV exposition and immunosuppression. MCC are most common in patients over 70 years and half of them manifest in the head and neck region. They early metastasize to regional lymph nodes. Surgical therapy should include wide resection of the primary tumor and diagnostic lymph node excision. In the head and neck region this means usually ipsilateral selective neck dissection. Adjuvant radiotherapy of the primary tumor bed and associated lymph nodes of the head and neck region decreases recurrence and should be performed in every patient regardless of the T- and N-stage. In the head and neck region adjuvant radiotherapy can only be spared in selected patients with low-risk profile (wide excisional margins > 2 cm, primary tumor size > 1 cm, absent lymphovascular infiltration, no immunosuppression and pathologic negative cervical lymph nodes). Isolated radiotherapy or systemic therapies are usually applied in patients with metastasized MCC. Disease recurrence is most common in the first two years after initial diagnosis. Patients should be examined at short intervals during this time.