The pulmonary artery sarcoma (PAS) is a rare malignant tumor of pulmonary artery (PA). The clinical symptoms and imaging findings are similar to those of pulmonary embolism, which is easily misdiagnosed. Here, we present a biopsie-confirmed case of primary PSA, who received target therapy with doxorubicin. This case highlights the importance of considering all relevant factors to make the correct diagnosis.