Pulmonary sequestration is an uncommon disease, accounting for only approximately 1.5% of all congenital pulmonary malformations. In most cases, the diagnosis is a result of accidental radiological findings; it is rarely accompanied by clinical symptoms, and is more commonly associated with other congenital malformations. Herein, we reported a case of pulmonary sequestration presented as massive left hemothorax and associated with primary lung sarcoma. A pneumonectomy via thoracotomy was attended with complete resection of sequestration and of sarcoma. The postoperative course was unremarkable, and the patient was discharged on postoperative day 11.