Primary endobronchial leiomyosarcoma is extremely rare, which is uncommon of primary endobronchial tumors. We report a primary endobronchial leiomyosarcoma. A 19-year-old male patient was admitted to the hospital ulcerative endobrochial tumor in the origin of left lower lobar bronchus and bronchoscopic biopsy showed a endobronchial leiomyoma. The patient underwent a left lower sleeve lobectomy and final pathologic diagnosis was ㅁ primary endobronchial leiomyosarcoma. After 4 months, follow-up bronchoscopy reveled local recurrence of a endobronchial leiomyosarcoma on a left main bronchus. A left completion pneumonectomy was perfomed and he was discharged without complications.