The Epstein-Barr virus related post-transplant lymphoproliferative disorder (EBV-PTLD) is one of the deadliest complications after hematopoietic stem cell transplantation in children. Multiple risk factors have been associated with the onset of EBV-PTLD such as reduced intensity conditioning (RIC), use of antithymocyte globulin (ATG), graft-versus-host disease (GVHD), cytomegalovirus reactivation, etc. There is no clear consensus on the treatment of EBV-PTLD in children transplant recipients due to few clinical trials and the rarity of the disease. This article aims to explore the entity of EBV-PTLD in childhood hematopoietic stem cell transplantation recipients, expanding on pathogenesis, risk factors, clinical diagnosis and recent treatment strategy, as such to highlight the gaps in knowledge needed for building a perfect treatment paradigm suitable for all pediatric patients.