Abstract
We present a case of a 78-year-old woman presenting with dyspnea and discovered to have an intra-tracheal tumor. Resection revealed this to be an adenoid cystic carcinoma (ACC) of the trachea, which is a very rare entity. She was treated with subtotal resection followed by radiation to the residual tumor, following which she relapsed six years later. Due to its indolent nature, she continues to be followed without any therapy. The biology, clinical features, and natural history of this rare tumor will be discussed.