Pulmonary artery sarcoma is a rare malignancy with poor prognosis. Lack of specific clinical manifestations, some patients are even confirmed postoperatively or at autopsy, that leads to the delay in treatment. Early diagnosis and radical surgical resection provide the possibility of prolonged survival. We retrospectively enrolled 13 patients diagnosed with pulmonary artery sarcoma at our hospital between 2015 and 2019. Their clinical, laboratory, radiological, and histopathological data were collected and analyzed. Published case series were also reviewed. Results show that, the median age of the patients was 53 years, with 6 (46.2%) males. The most common symptom is exertional dyspnea. Erythrocyte sedimentation rate and C-reactive protein were increased in 76.9% and 69.2% of these patients, while D-Dimer remained normal or elevated slightly. Metastasis was present at diagnosis in eight (61.5%) patients. Ten patients were diagnosed histologically: three were diagnosed after pulmonary endarterectomy, four by endobronchial ultrasound-guided transbronchial needle aspiration, two by percutaneous lung biopsy, and one by endovascular aspiration biopsy. Four patients underwent surgery and one is waiting for surgery. Nine patients received chemotherapy; and three of them received targeted therapy with anlotinib after chemotherapy. Two patients received anti-PD-1 monoclonal antibody. One patient died during endobronchial ultrasound-guided transbronchial needle aspiration. Two patients died 9 and 13 months after diagnosis, respectively; one refused invasive diagnostic procedures and died three months after clinical diagnosis. In conclusion, the most appropriate approach to get tissue specimen needs to be tailored to every pulmonary artery sarcoma patient. Pulmonary endarterectomy combined with chemotherapy and targeted therapy has prolonged their survival time.