The malignant lymphomas are a group of tumors arising in the lymphoreticular system. The lymphomas can arise in single lymph nodal groups or solitary extranodal tissues and organs. Nodal malignant lymphoma of the head and neck is a common clinical problem. Extranodal or organ lymphoma within the head and neck is, in contrast, quite rare. Although malignant lymphoma is frequently seen in various parts of the body, primary involvement of the larynx is extremely rare. When such a tumor is diagnosed in a nonlymphoid organ such as the larynx, the clinician is required to determine whether this is truly primary disease or merely the first clinical expression of a more general disorder. The authors experienced a case of malignant lymphoma of the larynx. A II-year old boy, whose chief complaint was progressive difficulty in respiration and swallowing for two to three weeks, was admitted to the hospital. We report this case with brief review of the literatures.