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Malignant peripheral nerve sheath tumors (MPNSTs), also known as malignant schwannomas, rarely occur in the mediastinum. These tumors have a poor prognosis. A PubMed search was conducted from 1950 to September 2016, and 57 cases of mediastinal MPNSTs were identified in the English-language medical literature. The symptoms, imaging findings, diagnosis, therapy, and prognosis of these 57 cases were analyzed. A total of 44 patients underwent surgical resection. However, 9 of the 57 patients had no evidence of disease. This review revealed that the main therapy for MPNSTs is complete surgical resection, but multimodal treatment including chemotherapy or radiotherapy plays an important role in improving survival.