Persistent and severe ST segment and T wave changes associated with an apical diastolic murmur were observed in an asymptomatic five-year-old girl for ten years. Recent development of exertional dyspnea and chest pain prompted a detailed cardiovascular re-evaluation. The symptomatology and the electrocardiographic changes are attributed to an angiomatous tumor involving the anterolateral myocardium demonstrated by selective coronary arteriography. The literature regarding primary cardiac vascular tumors was reviewed and the clinical and therapeutic implications were discussed.