Malignant lymphoma with a high content of epithelioid histiocytes is a rare non-Hodgkin lymphoma first described by Lennert and Mestdagh (Lennert's lymphoma) in 1968. Distinguishing characteristics include frequent involvement of the nasopharynx, Waldeyer's ring, and cervical lymph nodes. Of the four patients included in this report, three had Waldeyer's ring involvement and four had cervical lymphadenopathy. Our experience and that of others demonstrate the unpredictable and variable course of the disease, as well as the inconsistent response to various therapeutic modalities; especially interesting is the remission obtained in one patient treated with corticosteroids alone, in contrast to the others who followed a rapid downhill course to death in spite of (or as a complication of) chemotherapy and radiation. The role of the otolaryngologist in the diagnosis and management of the disease is emphasized.