Pulmonary blastoma is a rare malignant lesion with a poor prognosis. We described a case of a 47-year-old woman with a large biphasic pulmonary blastoma, involving the left pulmonary artery. Under cardiopulmonary bypass, it was treated with radical left intrapericardial pneumonectomy and pulmonary thromboendarterectomy. Subsequent chemotherapy and radiotherapy was used. Three years postoperatively, the patient was clinically and radiologically free of tumor.