Merkel cell carcinoma is a rare malignant neuroendocrine tumor of the skin with features of epithelial differentiation. It mostly arises in the head and neck region of elderly persons. Its biologically aggressive behavior is documented by a high rate of local recurrences and distant metastases and, therefore, demands an early and acurate diagnosis. There is still much confusion concerning proper diagnosis and therapy of this tumor. The article addresses established and controversial data about the histogenesis, clinical presentation, diagnosis, and treatment of Merkel cell carcinoma.