Laryngeal extranodal non-Hodgkin lymphoma is uncommon, accounting for less than 1% of all laryngeal neoplasms; the B-cell phenotype is predominant. Lymphomas outside the nasal cavity are rare and highly aggressive. We present a case of primary natural killer T-cell (NK/T-cell) lymphoma of the larynx that arose in a 45-year-old man. Because only a limited amount of data is available on laryngeal NK/T-cell lymphoma, the mainstay of treatment remains unclear, although some data suggest that radiotherapy alone is the best option. Our patient was treated with chemotherapy and radiotherapy, and he remained in remission 2 years later.