In the general population, Merkel cell carcinoma (MCC) is a very rare neuroendocrine primary skin cancer, known for its high propensity for local recurrence and distant metastases. Treatment for this neoplasm is individualized on the grounds of clinical staging at presentation, and may include surgical excision, radiotherapy and chemotherapy. Several studies suggest that MCC occurs more frequently and with a more aggressive course in immunocompromised patients such as organ transplant recipients and those infected with human immunodeficiency virus (HIV). A case of this cutaneous malignancy, characterized by a short-term local recurrence and systemic fatal spread in spite of surgical treatment, radiotherapy and chemotherapy, is described in a patient with advanced HIV infection.