Congenital aortic valve agenesis is a rare heart anomaly usually associated with other developmental cardiac malformations, part of the so–called ‘hypoplastic left–heart syndrome’. To the best of our knowledge, most reported cases of aortic valve atresia or agenesis have been associated with anomalies of the mitral valve, hypoplasia and/or coarctation of the ascending aorta, malformation of the left ventricle or the interventricular septum, patent foramen ovale and coronary abnormalities. These malformations are responsible for 25% to 40% of all neonatal cardiac deaths. We report a case of aortic valve agenesis associated with left ventricular fibroelastosis found in a 25–week miscarried fetus, product of the first pregnancy of two non–consanguineous healthy parents, with no history of drug abuse or exposure to toxic or teratogenic agents