A 66-year-old woman was admitted to our hospital because of left ventricular failure and nonsustained ventricular tachycardia. Two-dimensional echocardiography demonstrated prominent trabeculations and deep intertrabecular recesses, findings consistent with noncompaction of the ventricular myocardium. Myocardial perfusion scintigraphy demonstrated a defect in the anterobasal left ventricular segment. Coronary angiogram was normal, but the left ventriculogram showed an aneurysm in the anterior myocardial segments. This is the first reported case with isolated noncompaction of the ventricular myocardium associated with left ventricular aneurysm.