AbstractObjective:To review the existing diagnostic modalities and treatment for primary tracheal synovial sarcoma, and to report a case of primary cervical synovial sarcoma arising in the trachea.Design:Retrospective.Setting:Head and neck surgery unit at a tertiary university centre.Patient:One case of primary cervical tracheal monophasic synovial sarcoma diagnosed by SYT–SSX gene rearrangement.Intervention:This patient underwent surgical resection of the synovial sarcoma, together with tracheal resection and primary anastomosis assisted by laryngeal-releasing manoeuvres, without complication.Main outcome measures:Clinical, radiographical, pathological and surgical information were collected.Result:One year post-operatively, there was no evidence of recurrence.Conclusion:Synovial sarcoma arising in the trachea is very rare. Diagnosis is confirmed by demonstrating the SYT–SSX gene rearrangement. The first-line treatment is surgery.