Objectives: Posttransplant lymphoproliferative disorder following solid organ transplant is a life-threatening form of posttransplant malignancy. Its occurrence is typically associated with Epstein-Barr virus and profound immunosuppressive therapy. We describe a case of posttransplant lymphoproliferative disorder in the brain parenchyma, 4 years after renal transplant. Case Report: A 23-year-old man was evaluated for generalized headache 4 years after receiving a deceased donor renal transplant. After initial immunosuppression with tacrolimus and prednisolone, mycophenolate mofetil was added for maintenance immunosuppression. A tumor in the right occipitoparietal lobe was detected by magnetic resonance imaging and excised. Immunohistochemical testing of the tumor revealed B-cell marker and Epstein-Barr virus. After surgery, the dosage of immunosuppressive drugs was reduced, and the patient was treated with chemotherapy and radiotherapy. Our patient is well after treatment. Conclusions: Reduction in immunosuppressive therapy is an important component of treatment for Epstein-Barr virus-positive posttransplant lymphoproliferative disorder and may lead to remission in early disease. if reduced immunosuppression fails to control early disease, cytotoxic chemotherapy, surgery and radiotherapy, antiviral therapies, and cell-based therapies are other options for treatment.