OBJECTIVES:  Merkel Cell Carcinoma (MCC) is a rare, lethal cutaneous skin cancer with no approved drug therapies and limited treatment options. Our objective was to conduct a comprehensive review of MCC disease burden. METHODS:  We conducted a targeted literature review of studies published from 2010 to 2016 to assess epidemiology, patient burden, and unmet needs associated with MCC. An article was retrieved for full review if the abstract met each of the following criteria: reported incidence, natural morbidity or mortality of MCC; derived from a peer-reviewed journal; and reported in English-language published since January 2010. RESULTS:  The incidence of MCC (per 100,000 persons per year) is reported highest in Australia (0.82-1.60), followed by the US (0.6), Netherlands (0.35), Sweden (0.18-0.33), Finland (0.24-0.25), Spain (0.28), Denmark (0.22), South East Scotland (0.133), France (0.13). Inadequate data available from countries like Canada, UK, and Germany. Typically, stage I-II MCC is observed in approximately 60% - 75% patients, stage III in 10% - 30% patients, and stage IV (metastatic) in 2% - 16% patients. The 5-year and 10-year survival is 0% - 68%, and 21% - 65% respectively, based on factors such as disease stage and gender. Although less than 20% patients progress to advanced metastatic stage IV MCC, they experience the highest unmet need due to lack of effective therapeutic options. CONCLUSIONS:  The current treatment options usually include a combination of surgery, radiotherapy, and chemotherapy, but there is a significant need of newer, better treatments which can increase the survival. No evidence is found in the literature that reports the humanistic and economic burden related to MCC. Future research is warranted to adequately quantify the burden-of-illness of MCC and assess comparative effectiveness of evolving treatment options to better inform patients, prescribers and payer organizations concerning optimal modalities of disease management.