Angiosarcoma arising in a long-standing schwannoma is extremely rare and only a few cases were reported in the English literature. Besides tumors arising from vagus, sciatic or adrenal nerves, tumors growing on neck, foot or kidney were also described. To the best of our knowledge, in this article, we report the first mediastinal case occurring in longstanding schwannoma in a 53-year-old female patient. The patient was admitted to our clinic with severe dyspnea and palpitation. Her medical history showed a progressive right-sided paramediastinal mass which was first diagnosed in 2002. Three transthoracic needle biopsies performed in 2002, 2015 and 2016 were all non-diagnostic. An operation was suggested since 2002, but the patient has not accepted. Thorax computed tomography and magnetic resonance imaging revealed a huge mediastinal mass nearly fulfilling the right hemithorax. A diagnosis of "malign spindle cell tumor" was established with the last transthoracic biopsy and total surgical resection via posterolateral throcatomy was performed. Microscopically, tumor was composed of two components: a benign schwannoma and an epithelioid angiosarcoma. Endothelial and neural cell differentiations were confirmed immunohistochemically.