Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin’s lymphoma. Although progressive lymphadenopathy is a typical feature, extranodal involvement may also occur, including the gastrointestinal tract, skin, bone, thyroid, and testes. Central nervous system invasion is rare, so differentiating it from diseases such as inflammatory demyelinating disorder or infection is essential. DLBCL is therefore a challenge to diagnose, especially when the first findings are neurological symptoms. We report an unusual case of DLBCL that presented as transverse myelitis.