BACKGROUND Composite lymphoma is a rare entity where two or more distinct subtypes of lymphoma coexist within a single organ or tissue. METHODS We report a new case of a 67-year-old Caucasian male patient, who presented with fatigue, weakness, weight loss, and polyuria. He also had epigastric and left lumbar pain, enlarged spleen, and enlarged left axillary lymph node on examination, with no relevant medical or familial history. A biopsy from the node showed an appearance of T-cell rich, histiocyte-rich diffuse large B-cell lymphoma and nodular lymphocyte predominant Hodgkin lymphoma. The patient was initially treated with adriamycin (doxorubicin), bleomycin, vinblastine, dacarbazine chemotherapy regimen, then switched to rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone regimen. During the therapy, some regression was noticed, especially in the size of the splenic enlargement; however, the patient died 2 months after completing the regimen. CONCLUSIONS Composite lymphomas should continue to be studied. Also, treatment is still debatable in type, efficacy, and outcomes.