Introduction Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine malignant cancer. It is an epidermal cancer common in the head and neck. Objectives Though there is limited number of cases described in the literature for the treatment difficult to obtain. Our purpose was to present the clinical course and treatment of four patients with MCC. Methods We conducted a retrospective analysis and obtained detailed clinical information for all 4 patients treated for MCC at the ENT Department of the SS Annunziata Hospital in Chieti, Italy, from 2013 through 2015. Results In our study, two patients presented with the tumor in a rare site (lower eyelid). All of the patients underwent surgical treatment: three patients had free excision margins and negative sentinel lymph nodes (SLNs) while 1 patient had free excision margins and positive SLNs. The latter patient underwent ipsilateral neck dissection. In another patient, the fluorodeoxyglucose positron emission topography (FDG PET)/computed tomography (CT) performed 6 months after the surgery has shown high metabolic activity in the left parotid gland, and the patient underwent total parotidectomy and a neck dissection. Conclusion Sentinel lymph node biopsy is a useful technique in small size MCCs of the head and neck. However, the parotid gland should be strictly controlled in patients with lower eyelid tumors.