A primary pulmonary leiomyosarcoma is a very rare pulmonary malignancy that arises from smooth muscle of either the bronchial or arterial walls. Common symptoms of the tumor are cough, dyspnea, chest pain and hemoptysis. The diagnosis of a primary pulmonary sarcoma can be established only after extensive clinical and radiologic examinations have failed to identify an alternative primary source. The only effective treatment for the tumor is a complete surgical resection when feasible. The type of resection is dictated by the local anatomic extent of the tumor. We report a case of a 21-year-old male with a primary endobronchial leiomyosarcoma who presented with massive hemoptysis. A necrotic ulcerative endobronchial lesion was observed in the orifice of left lower lobe bronchus on a bronchoscopic examination. He was treated with a complete sleeve resection of the left lower lobe. Three months later, local recurrence of the tumor was noticed on the follow up bronchoscopy and a then left pneumonectomy was then performed. Fifteen months later, the patient died from empyema with a bronchopleural fistula that was associated with tumor recurrence at the stump of the pneumonectomy.