Merkel cell carcinoma (MCC) of the skin is a rare, aggressive form of skin cancer that metastasizes to other parts of the body. This cutaneous neuroendocrine tumour mainly affects older people, with most cases generally occurring over the age of 50 years. Merkel cell polyomavirus has been shown to induce gene mutations resulting in this skin cancer, with immunosuppression and ultraviolet radiation being other key risk factors in its pathogenesis. MCC is clinically seen as a rapidly enlarging, isolated, irregular erythematous nodule typically found on sun-exposed sites. Diagnosis is through clinical examination followed by tissue biopsy, which demonstrates characteristic histopathological neuroendocrine features. Immunohistochemistry plays a crucial role in diagnosis with the characteristic perinuclear staining with cytokeratin-20 helping to differentiate it from other morphologically similar tumours. Sentinel lymph node biopsy and imaging is essential for staging and determining prognosis. Surgical excision is the mainstay of treatment for localized disease although adjuvant radiotherapy is often required. Metastatic disease involves a very poor prognosis, and immune checkpoint inhibitors have recently shown promise in the treatment of metastatic disease. Avelumab, a monoclonal antibody that binds to the programmed death-1 receptor, has been approved by the National Institute for Health and Care Excellence and shown encouraging survival outcomes. It provides an option for treating metastatic carcinoma in adults after they have failed ≥ 1 line of chemotherapy for metastatic disease.