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Merkel cell carcinoma (MCC) is synonymous with primary cutaneous neuroendocrine carcinoma. It tends to affects elderly whites, but there is also an increased incidence among immunosuppressed patients. The recent identification of a novel polyomavirus associated with the tumor has stimulated renewed interest in its pathogenesis. MCC tends to show classic histologic features of a neuroendocrine carcinoma and is often positive for CK20, but nonclassic cytologic findings and unusual immunophenotypes may be observed and can lead to a diagnostic confusion. MCC needs to be distinguished from other primary cutaneous tumors with a small cell appearance and metastatic tumors. Surgical excision is the treatment of choice, but radiation therapy has also found to be effective. Sentinel lymph node biopsy has become an integral part of the staging of patients with MCC.