Abstract: Objective To study the clinical features, diagnosis and treatment of primary pulmonary artery tumor. Methods One case diagnosed with primary pulmonary artery tumor in our hospital in 2011 was enrolled in this study. The clinical characteristics, diagnosis, treatment and follow-up results of the patient were retrospectively analyzed and its related literatures were reviewed. Results The patient was a 47-year- old male. His main symptom was short of breath after doing activities. The enhanced CT showed a low-density mass in main pulmonary artery extending to bilateral pulmonary artery trunk, and pulmonary artery malignant tumor was diagnosed in the clinic. Due to the special location of the mass, the fine needle acupuncture biopsy could not be done for pathological diagnosis and surgical operation was not performed. The patient was treated with chemotherapy of docetaxel plus nedaplatin, radiotherapy, traditional Chinese medicine and so on. The symptoms of the patient were distinctly improved after careful treatments. An abdominal mass was found after 25 months’ treatment, and the postoperative pathology showed malignant spindle cell tumor which was considered from pulmonary artery tumor metastasis. The patient was followed up for survival in good condition until June 2014. Conclusion Primary pulmonary artery tumor is a rare disease, which is easily misdiagnosed as pulmonary embolism in clinical practice. Enhanced computed tomography can improve early diagnosis. The proven diagnosis depends on histopathological examination. Comprehensive treatment such as chemotherapy and radiotherapy shows solid therapeutic effects.