Abstract: Pulmonary artery sarcoma is a rare and lethal malignant tumor that typically affects pulmonary artery trunk.It arises from the intima or subintima of pulmonary artery trunk,also can be originated in the heart valve and the right ventricular outflow tract.The etiology of pulmonary artery sarcoma has been unknown.Its signs and symptoms are nonspecific and patients are often misdiagnosed with chronic pulmonary thromboembolism.In recent years,with the improvement of diagnosis technology,the understanding of the disease and the preoperative diagnosis rate increase.This paper reviews the diagnosis and treatment progress in the following.