The frequency of cardiovascular anomalies in patients affected by Turner's syndrome is well recognized. Its most frequent anomalies are aortic coarctation and a bicuspid aortic valve which are both found in 30% of cases. Other frequent anomalies are the dilatation of the ascending aorta, the aortic valve stenosis and/or insufficiency, the partial anomalous pulmonary venous drainage, and the interatrial septal defect. The aortic dissection and the aortic aneurysmal rupture, both complications of the cystic media necrosis of the aorta, are unusual, but dramatic, in their manifestations. The authors illustrate the case of a 6 year old female with Turner's syndrome by chance come under their observation for a cardiological evaluation according to which an azigos lobe and a bicuspid aortic valve. The authors consider it is the first time that an investigator has pointed out the association of these anomalies in Turner's syndrome. The authors believe that the illustrated case confirms the value of an echocardiographic evaluation as regards patients with Turner's syndrome, even with no findings of cardiovascular disease.