Posttransplantation lymphoproliferative disease is the most common malignancy in T-cell immunocompromised patients and often involves extranodal sites including the central nervous system (CNS). Primary posttransplantation CNS lymphomas are rare and, accordingly, are poorly characterized. Herein, for the first time, we describe the radiologic and pathologic features of a primary CNS posttransplantation lymphoproliferative disease that reveals Epstein-Barr virus-positive Hodgkin lymphoma. The lesion showed characteristic Hodgkin-Reed Sternberg cells with a typical immunophenotype, accompanied by numerous nonneoplastic B cells and T cells demonstrating classic Hodgkin lymphoma within the brain. The 65-year-old patient was treated by reduction of immunosuppression and cerebral radiation therapy and remains in remission 18 months after the initial diagnosis.