Primary lymphoma of the larynx is rare, accounting for less than 1% of all laryngeal neoplasm. Early symptoms are ambiguous and nonspecific, and confirmation of the diagnosis is often difficult. The aim of this study was to report the experience of our department in the management of these aggressive lesions, as they require special diagnostic and therapeutic attention. We enrolled 3 patients who diagnosis of lymphoma involving the larynx were retrospectively reviewed. The histopathological diagnosis revealed 1 case of diffuse large B cell lymphoma, and 2 case of NK-T cell lymphoma. Details of the presentation, diagnostic procedures, treatment, and outcome of these patients were presented. Primary laryngeal lymphoma is a rare entity. Early symptoms are subtle and nonspecific, and confirmation of the diagnosis is often difficult. Because of the rarity of this tumor type, the optimal management remains controversial and it seems that should be managed not as a distinct disease entity but as an unusual presentation of non-Hodgkin lymphoma, according to the recent treatment trends.