Merkel cell carcinoma (MCC) is an extremely rare skin cancer and was described in 1972. It characteristically grows rapidly and can be deadly by the time it is diagnosed. Surgery, in addition to radiation, is the mainstay of treatment, and awareness of the disease is key in improving clinical recognition. This chapter addresses risk factors such as UV exposure, diagnostic pitfalls such as the tumors phenotypic variability, and the care plan for a patient found to have MCC.