Epstein-Barr virus (EBV)-associated lymphoproliferative disorder is a well-known complication of solid organ transplantation. The risk factors include type and degree of immunosuppression and EBV status of the donor and recipient before transplantation. The manifestations of posttransplantation lymphoproliferative disorders are myriad, from subtle findings such as tonsillar hypertrophy to fulminant manifestations such as hepatocellular failure and death. Here we report an unusual presentation of posttransplantation lymphoproliferative disorder as unilateral leg swelling in a 68-year-old man 10 years after kidney transplantation. The patient had detectable EBV-DNA by polymerase chain reaction in the peripheral blood and EBV messenger RNA by in situ hybridization in biopsy tissue. The patient's tumor did not respond to reductions in immunosuppressive medication, intravenous immunoglobulin, ganciclovir, and rituximab. Eventually the patient responded to CHOP (cyclophosphamide, Oncovin [vincristine, Eli Lilly, Indianapolis, IN], prednisone, and doxorubicin) therapy. He is currently 2 years posttreatment, with a serum creatinine of 0.8 mg/dL, on no immunosuppression.