A 52-year-old man had been hospitalized in April 2006 as a result of right chest pain. Chest computed tomography had shown right pleural effusion and pleural thickening, and open pleural biopsy specimens had revealed as epithelioid-type pleural mesothelioma (PM). Chemotherapy had been performed, and we could control the disease progression for 5 years. However, in June 2011 the remarkable increase of pleural effusion and brain metastasis appeared. He died after five months despite treatment, and by autopsy we found the expansion of the primary lesion and extensive metastatic lesions (brain, peritoneum, pancreas, liver, spleen, and bone). Histological findings of brain and pleural lesions were sarcomatoid-type predominant PM. Considering a pathogenesis of PM, this is an interesting case, because intermingled sarcomatous components or epithelial mesenchymal transition might influence the disease progression.