Diffuse large B cell lymphoma (DLBCL), being one of the major subtypes of non-Hodgikin’s lymphoma, is marked by extranodal involvement. In patients with DLBCL, the stomach and gastrointestinal tract are commonly involved, but few cases have reported the involvement with the middle ear cavity and temporal bone. The present study reports a patient diagnosed with DLBCL characterized by middle ear cavity involvement. We also reviewed other similar cases in the literature.