Granular cell tumors are uncommon benign neoplasms. Their location is mostly in the head and neck region; appearance in other parts of the body is rare, but it has been reported. We present the case of a 14-year-old girl with a granular cell tumor of the trachea. The tumor was incidentally found at bronchoscopy performed to exclude suspected foreign body aspiration. It was located in the ventral part of the main carina. Biopsies revealed the histologic pattern of a benign granular cell tumor. The girl underwent resection of the main carina followed by reconstruction of a neo-carina with both main bronchi. She has not had any recurrence of the tumor during 3 years of follow-up.