Abstract
Abstract: An Epstein–Barr virus (EBV)‐seronegative 31‐year‐old male underwent cardiac transplantation in 1991 for congenital cardiomyopathy. He presented with a protracted course of waxing and waning lymphadenopathy beginning four years after transplantation with eventual progression to a fulminant EBV‐positive large cell lymphoma eight years after transplantation. Risk factors for the development of post‐transplant lymphoproliferative disease in this patient, the importance of a standardized approach to pathology in assessing therapeutic options, and the management strategies used are discussed.