Background: Cancers, and specifically lymphomas, are one of the main causes of morbidity and mortality after heart transplant. We encountered this complication on a 54-year-old male patient, which brought to our attention the lack of a specific and appropriate treatment for a primary cardiac lymphoma on the graft. Objective: This case report highlights the challenging management of primitive heart transplant lymphoma and its treatment. Method: electronic medical record literature reviewed through Pubmed interface of Medline. Result: We report the case of an Epstein-Barr Virus (EBV) induced primary cardiac lymphoma on the graft. The lymphoma spread quickly in spite of the numerous treatments we tried and resulted in rhythmic complication. Endocavitary implantable cardioverter defibrillator was implanted but quickly removed due to local complications. The patient died from a sudden death at home shortly after. Conclusions: Primary cardiac lymphoma on the graft is highly rare with poor prognosis and rhythmic complications. Further studies are needed to assess the benefits of other treatment approaches, such as radiofrequency ablation techniques.