Neurilemmoma is a benign and slowly growing neurogenic tumor. Intrathoracic neurilemmoma often develops in the chest wall and posterior mediastinum, but endobronchial neurilemmoma is extremely rare. The diagnosis of endobronchial neurilemmoma with preoperative imaging findings is challenging and is usually made via postoperative pathological examination. These authors encountered a case of primary endobronchial neurilemmoma in a 52-year-old woman who had no symptoms. A 3.0 × 2.6 cm mass in the right lower lobe projecting into the mediobasal segmental bronchus was shown in the results of the contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) of the chest. Benign neurilemmoma was confirmed via bronchoscopic biopsy, and surgical resection (sleeve bronchial excision and end-to-end anastomosis) was performed.