Background: Comprehensive data on cutaneous Epstein-Barr Virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS) is limited. Objectives: Systematic review of the clinicopathological features, immunophenotypes, and prognosis of cutaneous EBV-positive DLBCL, NOS Methods: We searched five databases and included studies with extractable data. Moreover, we searched the Asan Medical Center database for cases of cutaneous EBVpositive DLBCL, NOS and primary cutaneous EBV-negative DLBCL, leg type and compared the two diseases. Results: We included 22 cases of cutaneous EBV-positive DLBCL, NOS including 17 primary cutaneous. We also identified 21 cases of primary cutaneous EBV-negative DLBCL, leg type. Primary cutaneous EBV-positive DLBCL, NOS had more variable clinical morphologies than EBV-negative DLBCL, leg type. Disseminated skin lesions were more common and the frequency of T3 stage and CD30 positivity was significantly higher in EBV-positive DLBCL, NOS than in EBV-negative DLBCL, leg type. Histopathologically, of 17 cases, 9 and 8 cases showed polymorphic and monomorphic subtype, respectively. Primary cutaneous EBV-positive DLBCL, NOS tended to have worse prognosis than EBV-negative DLBCL, leg type. Conclusion: Cutaneous EBV-positive DLBCL, NOS has distinct clinicopathological features and may have worse prognosis than primary cutaneous EBV-negative DLBCL, leg type.