OBJECTIVE: Merkel cell carcinoma (MCC) is a rare cutaneous malignancy with a high recurrence and mortality rates. More than half of MCCs occur in the head and neck region. This paper aims to present a retrospective case series study of primary MCCs of the head and neck treated in our department over 12 years.STUDY DESIGN: Six patients were identified, and their characteristics, treatment modalities, and outcomes are reported. A critical review of the current literature is also included to provide up-to-date information on MCCs with special emphasis on treatment modalities and disease prognosis.RESULTS: Management of head and neck MCCs requires early and accurate diagnosis and includes surgery, radiotherapy, and/or combination chemotherapy. Accurate cervical nodal staging is of paramount importance before establishing the definite treatment plan.CONCLUSIONS: The results of both our case series and literature data review indicate that elective management of regional lymph nodes is recommended instead of an observation approach for patients with no identifiable disease in the lymph nodes (cN0). Because the majority of MCCs arise in the head and neck region, oral and maxillofacial surgeons are likely be the first professionals who will encounter this disease and should therefore be aware of the current diagnostic and treatment modalities.