We have treated a patient with malignant peripheral nerve sheath tumor (MPNST) for 7 years by interdisciplinary therapy. The diagnosis and treatment are reported with the clinical course so far. A 20-year-old man underwent surgical resection of a tumor on the left side of the chest in 1990, and a diagnosis of Askin tumor was made. In 1993, the disease recurred in the left axilla and was surgically resected. The surgical specimen was investigated histologically, and a diagnosis of MPNST was made instead of Askin tumor. Recently, a tumor began to grow in the left pleural cavity and was surgical resected. The tumor tissue contained both cells with oval and long spindle nuclei, which were intermingled with each other. Immunohistochemically, the tumor cells were positive for vimentin and some of them were positive for S100 protein. Because electron microscopy showed cytoplasmic processes on the tumor cell and basal lamina-like structures, a diagnosis of recurrent MPNST was made. Because this lesion resembled Askin tumor in its clinical manifestations and histological characteristics, differential diagnosis from Askin tumor was necessary. When a malignant soft tissue tumor arising from the mediastinum or chest walls is detected, immunohistochemical and electron microscopic studies should be performed. The diagnosis should be based on the overall findings including the location and clinical data.