Epstein-Barr virus (EBV) associated lymphoproliferative disease (LPD) comprises a wide spectrum of clinical and pathological features [1]. This variety is most systematically categorized in post-transplant lymphoproliferative disorders (PTLD) [2], in which subtypes are ordered according to disease progression from reactive polyclonal proliferation to large cell lymphoma. However, whether this categorization is applicable to LPDs other than PTLD is not well explored. Here, we present a nontransplant case of EBV-LPD that initially presented as a polyclonal self-limiting proliferation and later transited to large cell lymphoma. This transition was associated with the progression of lung cancer and its therapy. Our case demonstrates that stepwise progression of LPD is a feature that can be observed in non-PTLD cases of EBV-LPD.