RationaleCardiac angiosarcoma is a rare malignant tumor, for which only surgery has been proven to be effective to date. Currently there are no reports as to whether a postoperative regimen of ifosfamide, epirubicin, and recombinant human endostatin is effective.Patient concernThe patient presented to us with chest pain and dyspnea.DiagnosisEnhanced computerized tomography (CT) and positron emission tomography–computerized tomography (PET-CT) suggested pericarditis and an atrial perforation, but malignancy was suspected, so the patient underwent an operation to resect the tumor and repair. Pathology of the tumor reseccted at operation showed the tumor to be an angiosarcoma.InterventionAfter the surgery, the patient was stared on a paclitaxel chemotherapy regimen (135 mg/m2 once every 3 weeks). However, 2 cycles later, pulmonary and hepatic metastases were found. Chemotherapy was then changed to ifosfamide, epirubicin (ifosfamide 2000 mg/m2 days 1–3, epirubicin 70 mg/m2 days 1–2) and recombinant human endostatin (7.5 mg/m2 days 1–14) in 3 weekly cycles.OutcomeThree cycles later, follow-up showed that chemotherapy had delayed progression of the pulmonary metastases, but that the hepatic node was still growing. The patient has now survived 8 months post surgery and is still on follow-up.LessonsThis case shows us that operation on late stage cardiac angiosarcomas can alleviate a patient's symptoms; postoperative paclitaxel monotherapy was insufficient and ifosfamide and epirubicin plus recombinant human endostatin has a limited effect on late stage cardiac angiosarcoma. Studies with a larger sample size are needed for verification of these findings.