Composite lymphoma (CL) is a clinically rare entity, with unknown pathogenic mechanisms. Its diagnosis is quite difficult, and there is currently no proven treatment. In this article, we report a 64-year-old male patient presenting with swelling and mild pain in the bilateral cervical lymph nodes. Radiology revealed lesions only involved the neck. Histological examination showed that the tumor tissue had two components: mixed cellularity classical Hodgkin lymphoma (CHL-MC) and diffuse large B cell lymphoma (DLBCL).The final diagnosis was CL in the bilateral cervical lymph nodes composed of CHL-MC and DLBCL stage IA. The patient received six cycles of rituximab plus cyclophosphamide, pirubicin, vincristine, and prednisone (R-CHOP) and two cycles of rituximab maintenance therapy, and complete response (CR) was achieved. No progression was found during the 9 months of follow-up. We conducted a literature review of 28 cases of CL with CHL and DLBCL. The demography of the disease, the location of the disease involved, the subtype of CHL, the Epstein-Barr virus infection, treatment and survival were described in detail. In the discussion section, we analyzed the definition, pathogenesis, diagnosis, treatment and prognosis of CL. We hope that through case reports and literature review, we can improve the understanding of CL and optimize its treatment.