We report a case of lymphomatoid granulomatosis (LG), arising in a 60-year-old man in the setting of an acute myeloid leukemia. LG is a rare Epstein Barr virus (EBV) lymphoproliferative disorder, generally occurring in a context of immunodeficiency. Patients usually present with respiratory symptoms and bilateral pulmonary nodules. Histologically LG is characterized by an angiocientric and angiodestructive lymphoproliferation of B/EBV+ cells admixed with numerous reactive T cells. The differential diagnosis mainly includes pulmonary vasculitis and Hodgkin's lymphoma. The outcome of this lymphoproliferation is highly variable, ranging from an indolent process to an aggressive large cell lymphoma.