Isolated laryngeal lymphoma is a rare occurrence. We found a total of 14 previously reported cases. These plus our additional case are reviewed in order to make recommendations for diagnosis, therapy, and follow-up. Histologic classification and clinical staging of lymphomas have limited usefulness, because the tumor can change celltype during the clinical course of the disease. Once histologic proof of the tumor exists, adequate evaluation consistsof physical examination to detect adenopathy elsewhere, bone marrow biopsy, urinalysis, blood uric acid levels, liver biopsy, and liver and kidney function tests. The search must continue in the chest by X-ray, the retroperitoneal nodesby lymphangiography and the abdominal visera by contrast radiography. Radiation therapy is the best initial treatment for isolated laryngeal lymphoma. If the tumor does not respond to irradiation, then operation to remove the lesion is required. This tumor has a tendency to recur at distant sites many years later.As with all cancer patients life-long follow-up is required.