Primary intratracheal neurilemmoma is rare in children. Here, we reported a case of tracheal neurilemmoma in a 10-year-old girl. She had recurrent cough, dyspnea, and tachypnea. Magnetic resonance imaging revealed a soft-tissue mass was located on the right lateral posterior wall of the trachea. A multidisciplinary operation was performed. Immunohistochemical analysis demonstrated a benign neurilemmoma. Her clinical symptoms and signs improved. We reviewed the clinical features and surgical procedures of ten children with intratracheal neurilemmoma reported in literature. Radiographic techniques and bronchoscopy are helpful for diagnosis of the disease. Long-time follow-up is required.