We encountered one very rare case of primary adenocarcinoma of the trachea. The patient was a 72-year-old woman who was hospitalized immediately following significant dyspnea and mental confusion. A computed tomography scan of her chest revealed a 14x13x11 mm tumor in the trachea. After establishing artificial respiration, a tracheal tubular resection was immediately performed using percutaneous cardiopulmonary support (PCPS). A postoperative pathological examination led to the patient being diagnosed with primary adenocarcinoma of the trachea, and she was treated with adjuvant chemotherapy. Since then, for approximately 1 year and 6 months we have detected no relapse.