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Background Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine tumor of the skin. It is associated with advanced age, ultraviolet (UV) radiation, and Merkel cell polyomavirus. It has a predilection for the lymphatic system, but rarely spreads to the central nervous system. Case presentation A 71-year-old Caucasian man with a history of rheumatoid arthritis and MCC of the right lower eyelid and cheek presented with left-sided hemineglect and word-finding difficulty. Twenty months earlier he had undergone local excision of a 3 cm lesion with negative margins, negative sentinel lymph node biopsy, and external beam radiation. On presentation he was found to have a 6.3 cm mass in the right frontotemporal region. He underwent prompt resection, with pathological analysis consistent with metastatic MCC. He subsequently underwent stereotactic radiosurgery (SRS) and adjunctive immunotherapy with pembrolizumab. He has since tolerated the therapy well and is currently without neurological symptoms or evidence of recurrence. Conclusions Cerebral metastasis of MCC is a rare event and should be considered when a patient with a history of MCC presents with neurological symptoms. Optimal treatment regimens of these rare cases are unclear; however, prompt resection, stereotactic radiosurgery, and adjunctive immunotherapy have shown an initial positive response in this patient.

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