We report a case of endotracheal (ET) neurofibroma with resultant severe airway obstruction undergoing segmental resection of the trachea. After the extracorporeal membrane oxygenation system (ECMO) was set up handily for use and comprehensive monitoring system was established, ET intubation under fiberoptic bronchoscopy (FOB) was performed under mild sedation. Surgery and anesthesia proceeded uneventfully and she was discharged uneventfully. Thorough preoperative evaluation, comprehensively anesthetic planning, FOB-assisted ET intubation before induction of anesthesia were mandatory to achieve a safe and delicate anesthesia for such a patient. In critical situation, conduction of anesthesia after establishment of ECMO support would be another choice.