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Background  Malignant schwannoma of the upper mediastinum originating from the vagus nerve is extremely rare.    Case presentation  A 46-year-old female was admitted for a left cervical mass which was associated with both hoarseness and Horner's syndrome. Chest computed tomography showed a mass extending from the left upper mediastinum to the left supraclavicular area. A fine needle aspiration cytological examination suggested primary lung cancer stage IIIB large cell carcinoma. After administering induction chemo-radiotherapy, a complete surgical resection was performed. The tumor was found to involve both the left vagus nerve and the left sympathetic nerve. Histological examination of the resected specimen revealed the tumor to be malignant schwannoma.    Conclusion  Despite incorrect preoperative diagnosis, the multimodality treatment administered in this case, including induction chemo-radiotherapy and surgery, proved to be effective.