Mucosal melanoma of the head and neck (MMHN) is a rare malignant tumor associated with a poor prognosis. A retrospective study of case records of patients treated at our department between 1992 and 2010 was carried out. Thirteen patients were enrolled. The median age of the patients (3 males and 10 females) was 61 years (range 39-78). The median follow-up period was 48 months (range 10-115). Two common primary sites were the nasal cavity (8 cases) and sinonasal complex (5 cases). Ten patients (77%) received curative surgery. Chemotherapy was administered to 10 patients. In addition, lymphokine-activated killer (LAK) cell therapy was administered to 7 patients as adjunctive immunotherapy after the initial treatment course. The overall 5-year, cause-specific survival rate was 56%. Patients who received adjunctive LAK cell therapy had a survival rate of 67% at 5 years, while patients who did not receive adjunctive LAK cell treatment had a survival rate of 33%. MMHN is associated with a poor survival rate. The most common cause of death is distant metastasis. Surgery, radiotherapy and chemotherapy are common strategies for MMHN, but the control of metastasis is difficult. The use of immunotherapy remains uncommon for MMHN. However, from the viewpoint of a systemic disease, due to its high rate of metastases, immunotherapy using LAK cell treatment may contribute to the improvement of prognosis in patients with MMHN.