The patient was a 34-year-old man with a primary angiosarcoma of the heart. Initial admission was for cardiac tamponade. He was treated with preoperative chemotherapy and radiation therapy and then underwent orthotopic heart transplantation. The patient had an uneventful postoperative recovery, received two postoperative regimens of chemotherapy, and, at 33 months after transplantation, had no evidence of recurrence or metastasis. We propose that a more aggressive management of these patients with difficult conditions is warranted.