Extracutaneous glomus tumors are uncommon, and their occurrence in the trachea is rare. We present a case of a surgically resected glomus tumor of the trachea in a 56-year-old woman who presented with worsening dyspnea and cough. Bronchoscopy and computed tomography showed a polypoid tumor arising from the posterior membrane of the lower trachea just above the carina; the tracheal lumen was approximately 80% occluded. The patient underwent successful tracheal sleeve resection with primary reconstruction. The histological characteristics and immunohistochemical profile were typical for this tumor. The clinicopathological features of this unusual neoplasm are discussed, and the literature is reviewed.
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