Primary malignant cardiac tumors are very rare. Among malignant tumors, sarcomas occupy first place. In particular, primary cardiac osteosarcoma is extremely rare. To the best of our knowledge, only 42 cases have been reported worldwide. Cardiac malignant tumors usually require complex operations due to the difficulty in completely removing the tumor with acceptable free surgical margins and because of the proximity to vital structures. The current multimodality treatment strategies for cardiac sarcoma are still suboptimal, and surgery in particular frequently has unsatisfactory results. We report a case of recurrent primary cardiac osteosarcoma in a young male who underwent trans-sternal right pneumonectomy and a wide resection of the left and right atrium followed by reconstruction with heterologous pericardium under extracorporeal circulation. The patient died 6 months after the operation due to local and systemic disease recurrence.