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Primary cardiac angiosarcoma is rare, but it is the most aggressive type of cardiac malignant tumor. When disease is confirmed it is often late in its stage resulting in a poor prognosis.We reported a 52-year-old woman who complained of dyspnea for 2 months and dry cough for 10 days. Computed tomography (CT), 18F-fluorodeoxyglucose (18F-FDG), positron emission tomography-computed tomography (PET-CT), and transthoracic echocardiography showed a space-occupying lesion 7 cm × 5 cm × 5 cm in the right atrium. Malignant cells can be seen in pericardial fluid by pericardiocentesis in local hospital, but not be diagnosed definitely. We conducted CT-guided biopsy of the tumor and observed the necrotic tissues. An exploratory thoracotomy was performed and histological examination confirmed angiosarcoma. Unable to be surgical excision due to infiltrating widely, the patients received chemotherapy with docetaxel for 2 cycles and the disease was progressive. Antiangiogenesis-targeted therapy was combined with chemotherapy for 3 months. Unfortunately, the tumor was still in progress. She died of respiratory failure, survived for 10 months.It is difficult to diagnose the primary cardiac angiosarcoma in early stage. Chemotherapy and target therapy are very limited in this case. Surgery remains the preferred treatment for patients with primary cardiac angiosarcoma. More efforts should be carried out on the effective diagnosis and multimodality treatment for this malignant disease.