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Lymphomatoid granulomatosis is a very rare B cell lymphoproliferative disease associated with Epstein-Barr virus infection. It is related to states of immunosuppression and affects the lung in more than 90% of cases, forcing the clinician to establish a differential diagnosis with other diseases such as infections, Wegener's granulomatosis, lymphoma, or lung metastases. There is no standard treatment for this disease. In this paper, we describe a rare case of a patient with grade 3 lymphomatoid granulomatosis with newly diagnosed HIV inf