A 40-year-old man with haemoptysis and serious spontaneous hemothorax. Chest computed tomography showed multiple flakes in his lungs, nodules shadow, right pleural effusion with right pulmonary atelectasis. The pathological specimens obtained by thoracic surgery revealed pulmonary angiosarcoma. Primary pulmonary angiosarcoma is a rare malignant tumor and is lack of typical clinical symptoms and imaging features, diagnosis mainly depend on histopathologic features and immunohistochemical analysis. There is no effective therapy for pulmonary vascular sarcoma. Integrated treatment can may extend the survival time of patients.