Primary lymphoma presenting a solitary lesion of the chest wall is extremely rare, as the majority of chest-wall tumors arise from metastasis. We report a case of a 64-year-old man with no history of HIV infection or pyothorax who presented with dry cough, right pleuritic pain and dyspnea. A computed tomography scan revealed an irregular pleural mass invading his right chest wall with pleural effusion. CT-guided needle biopsy revealed extranodal marginal zone B-cell lymphoma. The patient was treated with chemotherapy and radiotherapy. The patient has 9 years of follow up with 2 relapse's episodes.