Primary pleural lymphoma is a very rare neoplastic disease that originates from leukocytes within the pleural lining. Its exact cause is not well understood; however, chronic inflammation related to occupational exposures, autoimmune diseases, and infections has been suggested as a possible cause. While radiologic imaging is not diagnostic, it can offer insights into the disease process. Chest computed tomography usually shows unilateral irregular pleural thickening or nodularity, sometimes accompanied by a pleural effusion. Diagnosis depends on pleural biopsy, as pleural fluid cytology is often negative. Video-assisted thoracoscopic surgery or thoracoscopy with biopsy provides the highest yield for tissue sampling. Immunohistochemical staining is essential to determine the specific lymphoma subtype. Treatment generally involves chemotherapeutic regimens.