A 64-year-old man was admitted to his local hospital complaining of dyspnea, which began in spring 2009, during exercise. He was diagnosed with old pleural thickness, though a chest X-ray and CT scan revealed pleural thickness and effusion. Lumbago developed in December the same year, at which point chest-abdominal CT showed diffuse right pleural thickening and nodular lesions in the liver, and lumbar MRI showed pressure fractures of the lumbar vertebrae. A percutaneous pleural biopsy was performed, but no final diagnosis was reached. During the pleural biopsy under video-assisted surgery a thoracoscope revealed tumor cells with a storiform pattern, which led to the diagnosis of stage IV desmoplastic malignant pleural mesothelioma (cT4N0M1). Chemotherapy with cisplatin and pemetrexed and second-line treatment with gemcitabine were performed. However, the treatment was not effective in reducing the tumor, and the patient died as a result of tumor progression. We are reporting this case because desmoplastic malignant pleural mesothelioma is one of the most difficult malignancies to diagnose and also a very rare pathological subtype of malignant mesothelioma.