The aim of this study was to perform a systematic literature review of Merkel cell carcinomas (MCCs) originating exclusively in the ear, nose and throat (ENT) district. An appropriate string was run on PubMed to retrieve articles dealing with ENT presentations of MCC. A double cross-check was performed on citations and full-text articles found using the selected inclusion and exclusion criteria. In total, 43 articles were finally included in the study, describing 51 cases of MCC involving the ENT region: 22/51 (43%) cases involving the ear; 20/51 (39%) cases involving the mucosal sites; 9/51 (18%) cases involving other ENT regions. Patients with mucosal site involvement showed a higher mortality rate from the disease (45%) in comparison with the other two groups, especially when compared with those patients having primary involvement of the ear (22%). The ENT specialist should suspect and consider MCC, especially in elderly patients presenting with a suspicious lesion of the auricular pavilion, so as to avoid misdiagnosis and delayed treatments.