Primary bone lymphoma (PBL) is a peculiar extranodal presentation of non-Hodgkin's lymphoma. Primary bone diffuse large B-cell lymphoma (DLBCL) is the most common pathological type, comprising about 80% of PBL. The diagnosis of PBL depends on the combined clinical examination and imaging studies and is confirmed with immunohistochemical examination. Due to the rarity of this disease, more relative studies and case reports are needed to provide insight into this obscure lymphoproliferative malignancy. Here, we report one rare case of primary bone DLBCL involving the axial skeleton in a 37-year-old female.