An extremely rare case of malignant glomus tumor originating in the superior mediastinum was evaluated immunohistochemically and ultrastructurally. A 78-year-old woman who had been suffering from dysphagia and dyspnea had poorly-defined soft tissue mass, 4.5 x 2.5cm, in the superior mediastinum with direct invasion into the esophagus, trachea, and bilateral thyroid glands. This case is believed to be unique in several respects. There were neither recognizable findings of benign glomus tumor nor sarcomatous areas, in contrast to the previously reported cases. A definite direct invasion into the surrounding organs was identified. We therefore interpreted this case as primary malignant glomus tumor, not as glomangiosarcoma arising in a benign glomus tumor.