Merkel cell carcinoma (MCC) is a rare and aggressive neuroendocrine skin carcinoma. The purpose of this study is to describe clinical and pathological characteristics, diagnostic procedure and treatment outcomes of the patients with MCC of the head and neck treated in Otorhinolaryngology, Head and Neck Surgery Department of the University Hospital Center Zagreb between the years 2007 and 2011. Three patients with MCC of the head and neck were treated during this period. First patient was diagnosed with MCC of the left retroauricular region with metastases in the lymph nodes of the left side of the neck, pT2N2M0 Stage IIIB. Second patient was diagnosed with anaplastic carcinoma of the lower lip, pT1N1bM0 Stage IIIB and third patient was diagnosed with MCC of the face which was previously treated as benign lesion, cT1N1bM0 Stage IIIB. Two of the patients had second primary tumor of different histology. All of the patients were treated with wide surgical excision of the tumor and neck dissection combined with adjuvant radiotherapy. Treatment outcome was poor and reason for this was late detection of disease. Menagment of the MCC patients requires multidisciplinary approach with high clinical suspicion of the treating specialist and pathologist due to immunohistochemical techniques required for diagnosis. Detection of the MCC in earlier stages is necessary for the better survival rate.