A 34-year-old woman was admitted with a history of syncope and a mass was detected in the right atrium (RA) by transthoracic echocardiography (TTE). Preoperative chest computed tomography (CT) also demonstrated an RA tumor measuring 4×3 cm. We performed resection of the RA tumor under cardiopulmonary bypass. Histopathological findings showed that the tumor was an angiomyolipoma. It is well known that angiomyolipomas are most frequently found in the kidney and are associated with tuberous scleroses. There was no evidence of tuberous sclerosis in this case. Primary tumors of the heart are rare. However, there have been a few intracardiac angiomyolipomas reported previously.