Background: Lymphomatoid granulomatosis (LYG) is a rare and atypical EBV induced B-cell lymphoproliferative disorder. Clinical manifestations are mainly respiratory, with nodular infiltrates, varying in number and size being responsible for respiratory distress. Cutaneous, hepatic, or neurological involvement is also possible with the disease. Lymphatic involvement is uncommon. Although pathogenesis is not clearly elucidated, quantitative or qualitative cellular immunodepression is thought to be a main factor. Here, we report a case of concomitant LYG and pulmonary tuberculosis in an elderly woman. Case presentation: An 80-year-old woman presented to the emergency unit for steadily increasing dyspnea, with a workup revealing bilateral nodules and mediastinal lymph node enlargement on chest imaging, associated with a mildly elevated C-reactive protein (CRP). She had no relevant prior medical history and no known immunodepression. Empiric antibiotic therapy was initially started with amoxicillin-clavulanate, which was later combined to azithromycin following respiratory deterioration. A CT-guided lung biopsy showed grade I lymphomatoid granulomatosis. Treatment with corticosteroids and weekly rituximab was initiated, leading to a rapid improvement of respiratory symptoms. After the second dose of rituximab, initially collected sputum cultures were found positive for Mycobacterium tuberculosis. Rituximab was suspended and antituberculous treatment was initiated. Rituximab was restarted once tuberculosis was controlled. Follow-up imaging later showed adequate control of both infectious and hematological diseases with at least a partial remission of the lymphomatoid granulomatosis. Conclusions: This case report highlights both diagnosis and therapeutic challenges in a rare but plausible situation. Lymph-node involvement in lymphomatoid granulomatosis should always be questioned in LYG as it is seldom seen in the disease. Finally, pathologic confirmation for LYG should not eliminate the need for a comprehensive workup to eliminate other differential diagnoses with management-changing potential.

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