In this article, we reported the successful treatment for an 87-year old male suffering from discordant lymphoma. He initially presented with enlargement of left parotid lymph node in 1987. Then in 1988, he was diagnosed with small B-cell non-Hodgkin’s lymphoma through lymph node biopsy, and treated with 50 courses of cyclophosphamide, vincristine, prednisone (COP or OP), and short-term local radiotherapy. However, he relapsed in 2014 with a sore throat, and an epiglottis biopsy revealed anaplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma. The final diagnosis of the patient was sequential discordant lymphoma consisting of small B-cell non-Hodgkin’s lymphoma and ALK-negative anaplastic large cell lymphoma. Discordant lymphoma is rare, especially those involving both B cell and T cell. Biopsy for the tissue specimen of every lesion can help avoiding missed diagnosis, and thus choose a more appropriate treatment regimen.