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Multiple myeloma (MM) is characterized by abnormal proliferation of neoplastic plasma cells. Pleural effusion as an initial presentation of this disease is rare, as is true pleural myeloma. We herein present a case of solitary pleural myelomatous lesion in a 70-year-old male patient diagnosed by pleural biopsy via semi-rigid thoracoscopy followed by histopathological examination. Furthermore, a review of the related English literature identified 22 cases of pleural myeloma, only 3 of which were diagnosed by video-assisted thoracoscopy. To the best of our knowledge, this is the first reported case of a solitary pleural myelomatous lesion diagnosed by pleural biopsy via semi-rigid thoracoscopy. Patients with MM with pleural involvement, including the present case, appear to have a short survival despite aggressive treatment. Our patient received chemotherapy with bortezomib, epiadriamycin and dexamethasone; however, he deteriorated rapidly after one cycle of chemotherapy and succumbed to the disease 8 weeks after the initial presentation. According to our experience, semi-rigid thoracoscopy is an effective and safe method for obtaining a pleural specimen for histopathological evaluation.