Cardiac leiomyosarcoma and atrial intramural hematoma are extremely rare. Here, we present the case of a 52-year-old woman with dyspnea. Echocardiography showed a large mass in the left atrium. Tumor resection was performed. A pathological diagnosis of intramural hematoma was made. Ten months later, she presented to our hospital again with dyspnea. Echocardiography and computed tomography showed recurrence of the left atrial tumor. Another tumor resection operation was performed. The tumor originated from the inferior wall of the left atrium, and it was surrounded by atrial wall thickening. Complete resection was not possible. Although postoperative chemotherapy was prescribed, the patient died of heart failure nine months after the second operation.