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Abstract: Background: Primary pulmonary lymphoma (PPL) is a rare form of non-Hodgkin lymphoma (NHL) originating in the lungs, accounting for less than 1% of all NHL cases and under 0.5–1% of primary lung malignancies. Among its subtypes, primary pulmonary follicular lymphoma is particularly uncommon and may present diagnostic challenges due to its nonspecific respiratory symptoms. Aim: To highlight the clinical presentation, diagnostic workup, and therapeutic approach in a rare case of primary pulmonary follicular lymphoma, with emphasis on bilateral pleural effusion as the initial manifestation. Case Presentation: A 74-year-old male presented with progressive shortness of breath over several weeks without constitutional symptoms or prior pulmonary disease. Imaging revealed bilateral pleural effusions. Initial thoracentesis and cytology were non-diagnostic. Due to worsening symptoms, repeat thoracentesis was performed. Flow cytometry and FISH analysis of pleural fluid revealed monoclonal B-cell population positive for lambda CD10, BCL2, BCL6, and t(14;18)/IGH-BCL2 translocation—findings consistent with follicular lymphoma. Conclusion: This case underscores the importance of considering PPL in the differential diagnosis of unexplained pleural effusions. Despite initial negative cytology, advanced diagnostic tools such as flow cytometry and molecular studies are critical in reaching a definitive diagnosis. Early identification and targeted treatment are essential to optimize patient outcomes in rare presentations of NHL.