Natural killer/T (NK/T) -cell lymphoma in the larynx is extremely rare, as only 29 cases have been previously reported in the English-language, Japanese-language, and Chinese-language literature. Its characteristics have never been systematically illustrated. We report 2 new cases of laryngeal NK/T-cell lymphoma, and we discuss the process of the diagnosis of this disease, the choice of treatment, and treatment outcomes. We also summarize all 31 cases reported thus far. Symptoms of laryngeal NK/T-cell lymphoma are difficult to differentiate from those of other laryngeal diseases. The most common laryngeal subsite in the reported cases was the supraglottis, and roughly one-third of these cases involved the cervical lymph nodes. Because of our limited experience with this disease and the difficulties encountered in interpreting the pathologic findings, most patients required multiple biopsies over a few months before their diagnosis was confirmed. The outcome of treatment was generally poor. Radiotherapy, alone or combined with chemotherapy, was superior to chemotherapy alone in treating this disease in its early stages. In view of the frequency of local lymph node metastasis, irradiation fields should cover the entire cervical area. We believe that prompt diagnosis and treatment with radiotherapy are both critical to improving survival for patients with laryngeal NK/T-cell lymphoma.