A 9-year-old girl was admitted because of recurrent cough, dyspnea, and tachypnea for over 3 months, which had become worse for the preceding week. A chest X-ray revealed a mass in the lumen of the trachea. Fiberoptic bronchoscopy confirmed a polypoid mass in the upper trachea 3 cm under the vocal cords, with a 1.5-cm diameter that occluded approximately 70% of the lumen. Endoscopic excision of the neoplasm was performed with rigid bronchoscopy Results of immunolabeling, which was positive for S-100 protein and vimentin, demonstrated a benign neurilemmoma. Clinical symptoms and signs improved after the tumor was removed. Intratracheal neurilemmoma is an extremely rare neoplasm, and only 34 cases were previously reported worldwide. The most common presentation is airway obstruction. Preoperative diagnosis is difficult, and radiographic techniques are helpful for diagnosis. Bronchoscopy is recommended for diagnosis, biopsy, and resection of the tumor. Long-term follow-up after simple enucleation is required.