A 74-year-old man was admitted to our hospital with a cough. His chest X-ray film, chest CT scan and MRI showed a tumor of the chest wall. Histology of a percutaneous needle biopsy revealed malignant fibrous histiocytoma (MFH). The tumor enlarged rapidly, and the patient underwent resection including the 6th, 7th and 8th ribs and partial resection of the right diaphragm and the right middle and lower lung lobes. Multiple pulmonary metastases were found 40 days after the operation, and the patient died of respiratory failure 4 months after surgery. Although MFH is one of the most common soft tissue sarcomas, lesions arising from the chest wall are uncommon. We stress the need for early diagnosis and aggressive surgical resection in the treatment of MFH arising from the chest wall.