Primary tumors of the heart are rare. In the literature operations for heart tumors account for 0.3% of all cardiac surgical procedures. We report a very rare case of primary cardiac angiomyolipoma. A 39-years-old man was admitted with chest discomfort. A mass was detected in the left ventricular myocardium by enhanced chest computed tomography. We performed resection of the intramyocardial tumor under cardiopulmonary bypass. Pathologic examination revealed a 4×3×3 cm, dome-shaped, solid tumor composed of a mixture of blood vessels, smooth muscle, and fat tissue. Histopathological findings showed that the tumor was angiomyolipoma. Angiomyolipoma is a benign tumor and usually develop in the kidney and are associated with tuberous sclerosis. In our case, there was no evidence of tuberous sclerosis.