We describe the case of a patient with mildly dilated idiopathic cardiomyopathy and left ventricular aneurysm, diagnosed in absence of a prior clinical history and anatomo-pathological features of myocardial infarction. To ascertain the diagnosis of idiopathic cardiomyopathy, the patient underwent cardiac catheterization with coronary angiography, that showed the lack of epicardial artery stenosis and a slow run-off of the contrast. An endomyocardial biopsy showed the presence of hypertrophic myocytes and interstitial fibrosis. Moreover, a thoracic high resolution computed tomography showed the features of pulmonary bilateral basal emphysema, interstitial thickening and bronchiectasis. Alfa1-anti-trypsin plasma levels were reduced. The patient, because of worsening of clinical and hemodynamic conditions, underwent at age of 36 a combined heart-lung transplantation. The pathological examination of the native organs confirmed the previous diagnosis. At the moment, this is the second report in the literature concerning the presence of left ventricular aneurysm in a patient with idiopathic cardiomyopathy without an underlying coronary artery disease or prior history of myocardial infarction.