Thymomas generally grow applying pressure to the adjacent organs but they may infiltrate the capsule and extend directly to them. Invasion of the airway is extremely rare and generally related to high-grade thymomas. Few cases of thymoma with endobronchial polypoid growth and only one patient with tracheal invasion have been reported. We present a typical type A thymoma with endoluminal growth almost completely obstructing the trachea. A 93-year-old man was referred to our hospital with severe dyspnea and wheezing. He already presented a diagnosis of type A thymoma, discovered occasionally 8-year before and followed up with a yearly computed tomography (CT) scan. The CT scan of the thorax at admission, revealed a bulky anterior mediastinal tumor with an endotracheal growth, almost obstructing the airway. The fiberoptic bronchoscopy confirmed severe tracheal stenosis caused by a vegetating endoluminal tumor. Due to the severity of symptoms, we performed an emergency rigid bronchoscopy aimed to relieve the upper airway. The endotracheal tumor had a relatively small base of implant and was totally removed with mechanical resection. Pathological examination revealed a proliferation of oval or spindle neoplastic cells with bland nuclei and inconspicuous nucleoli and very few immature lymphocytes. Tracheal invasion by type A thymoma was finally diagnosed. No clinical evidence of endotracheal recurrence was recorded after a 15-month follow-up.