The authors report the case of a 15 month-old infant admitted to the intensive care unit for cardiogenic shock due to rupture of an aneurysm of the right anterior sinus of Valsalva into the left ventricle, associated with massive aortic regurgitation. The patient underwent a Ross procedure with resection of the aneurysm. This is a very rare condition because rupture of the aneurysm into the left ventricle has not been previously reported in a young girl to the best of the authors' knowledge. Moreover, a Ross procedure has not been previously described in this indication. The case illustrates the diagnostic difficulty associated with this pathology and underlines the necessity of thinking of this diagnosis in infants with appearances of dilated cardiomyopathy and aortic regurgitation.