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Primary pulmonary lymphoma is a rare entity. Furthermore, simultaneous bilateral spontaneous pneumothorax (SBSP) is a very rare condition which is often related to therapeutic complications. We present, to the best of our knowledge, the first case of primary pulmonary mucosa associated lymphoid tissue (MALT) lymphoma revealed by SBSP. A 50-year-old female was diagnosed with organizing pneumonia. One month later, she presented with sudden chest pain and shortness of breath due to SBSP. Bilateral chest tubes were inserted. A scan- guided right lung biopsy led to the diagnosis of primary pulmonary MALT lymphoma. The patient was treated with R-CHOP chemotherapy. The association between lymphoma and pneumothorax is extremely rare, often related to therapeutic toxicity. We report the case of SBSP as the first manifestation of primary pulmonary MALT lymphoma.

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