Merkel cell carcinoma (MCC) is a rare, aggressive neuroendocrine cutaneous malignancy with a high risk of local recurrence, regional spread, and distant metastasis[1]. The incidence is increasing, with an annual incidence of approximately 0.7 per 100,000 persons in the US and about 3,200 new cases being diagnosed annually[2][3]. Five-year survival depends on stage: approximately 79% for localized disease, 66% for regional involvement, and 31% for distant metastases[2]. About half of primary tumors are located in the head and neck region; tumors in this location have a worse prognosis and are more often negative for Merkel cell polyomavirus (MCPyV)[1][2]. Basic treatment for clinically node-negative disease includes wide local excision and sentinel lymph node biopsy followed by postoperative radiotherapy in case of a positive finding or adverse risk factors[1]. For more advanced stages, multimodal treatment (excision, nodal dissection, radiotherapy) is used, and immunotherapy with checkpoint inhibitors (eg, avelumab, pembrolizumab) has been shown to be useful and is included in the recommendations, including neoadjuvant use[1]. Chemotherapy is no longer recommended as standard treatment in the non-metastatic stage, as it has not been shown to improve overall survival[1].