Merkel cell carcinoma is a rare but aggressive neuroendocrine skin cancer, primarily affecting older, fair-skinned immunosuppressed individuals with significant ultraviolet sun exposure. Approximately 80% of cases are linked to Merkel cell polyomavirus (MCPyV), contributing to oncogenesis through viral genome integration and immune evasion. Clinically, MCC is a rapidly growing, painless nodule, often on sun-exposed areas, with a high propensity for lymphatic and distant metastasis. Diagnosis is based on histopathology (small, blue round cells, CK20-positive) and the AEIOU criteria. Management includes wide local excision with sentinel lymph node biopsy (SLNB), followed by adjuvant radiation therapy in high-risk cases. Platinum-based chemotherapy is utilized in advanced disease, but immunotherapy with PD-1/PD-L1 inhibitors has shown superior outcomes. Prognosis depends on tumor size, lymph node involvement, and immune status, with MCPyV-positive tumors showing a better outlook. Due to high recurrence rates, close follow-up is essential, alongside supportive care for symptom management and palliative treatment in advanced cases.