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Abstract Background Neurogenic tumors of the tracheobronchial tree are extremely rare and include neurofibroma and schwannoma. We report a case of primary recurrent tracheal schwannoma causing obstructive airway symptoms. Case presentation A 60-year-old man presented with obstructive airway symptoms due to recurrent tracheal schwannoma. Due the recurrence, size of the tumor and low surgical risk, the patient was treated with tracheal resection. Conclusion Primary endotracheal neurogenic tumors are extremely rare, but one should consider them in the differential diagnosis of persistent upper airway symptoms. While endoscopic therapies recur nearly a quarter of the time, surgical resections do not have any recorded recurrences.