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Lymphomatoid granulomatosis is a rare lymphoproliferative disorder composed of rare-to-abundant atypical Epstein Barr virus infected B-cells admixed with numerous reactive T-cells. We report a case of a 42 year-old man presenting with fevers of unknown origin and acute renal failure. CT scan demonstrated lung opacities which progressed to numerous nodules throughout both lungs without any cavitations. Wedge lung biopsy showed nodular polymorphous mononuclear infiltrates containing scattered atypical large Epstein Barr virus positive B-cells consistent with lymphomatoid granulomatosis. The patient responded to chemotherapy, but later underwent relapse and transformation to diffuse large B-cell lymphoma. The clinical and histological features of lymphomatoid granulomatosis and differential diagnoses as related to this case are discussed.