A patient had undergone surgical resection twice for primary and metastatic dedifferentiated liposarcomas. Follow-up computed tomography revealed a new tumor mass located at the cavoatrial junction. Prompt surgical resection of the tumor with thrombectomy was successfully performed using cardiopulmonary bypass with hypothermic circulatory arrest. We report a rare case of a patient surviving for 8 years after the first tumor resection despite the poor prognosis of metastatic or recurrent liposarcoma.