Epithelioid angiosarcoma arising in schwannoma is an extremely rare mesenchymal tumor that accounts for only 1 to 2% of all sarcomas. This type of tumor occurs in all parts of the body, most often in the skin and soft tissues and rarely in the mediastinum. The present study describes the case of an asymptomatic, 58-year-old male who presented with epithelioid angiosarcoma in the posterior mediastinum during a physical examination. Enhanced computed tomography of the chest revealed a 3.5 × 3.1-cm mass in the posterior mediastinum. Thoracoscopic mediastinal mass resection was performed under general anesthesia due to the possibility that the tumor was malignant. Pathological examination revealed the presence of angiosarcoma and schwannoma components. Immunohistochemical staining for cluster of differentiation (CD) 31, CD34, early growth response (EGR), vimentin, Sry-related HMG box (SOX)-10 and S-100 was strongly positive. The patient recovered and was discharged on postoperative day 5. Two months postsurgery, the patient returned for evaluation, and no evidence of tumor recurrence was observed.