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Spindle cell sarcomatoid carcinoma of the trachea is a rare tumor with an unclear clinical outcome. So far, only 3 cases of this tumor have been reported in English literature. Herein we report an Asian case of primary spindle cell sarcomatoid carcinoma in the cervical trachea. Case report: a 50-year-old Asian male presented with over 2-year history of an intermittent cough and worsening dyspnea. Workup revealed an irregular new growth in the mid-trachea, occupying more than 70% in the cross-section of the tracheal lumen. Specimen examination suggested some poorly differentiated spindle cells. Patient received surgical resection of the tracheal tumor followed by end-to-end suture. At 12 months, the patient is disease-free. Conclusion: Primary spindle cell sarcomatoid carcinoma of the trachea is extremely infrequent. In general, the optimal strategy is unclear in those patients. However, we suggest surgical resection as an initial strategy