Primary pulmonary artery sarcoma is extremely rare and a lethal tumor. Complete resection of the tumor and following adequate reconstruction is the only hope for cure and prolonged survival. We report a case of successful surgical excision of primary pulmonary artery sarcoma involving pulmonary trunk, pulmonary valve and right ventricular outflow tract wall, which were replaced with prosthetic valved conduit. Postoperative histopathological diagnosis was leiomyosarcoma. The patient is well without any evidence of recurrence at 10 months after surgery.