In 1888, Etienne Fallot described the last two components of Fallot’s tetralogy: right ventricular hypertrophy and dextroposition of the aorta over septal defect. Since 1671 the obstruction to the right ventricle outlet tract and the ventricular septal defect had been described. The presence of these four elements, together with another structural alteration such as the atrial septal defect, turns the tetralogy into Fallot’s Pentalogy. A case of a patient suffering from this condition is presented in this article, as well as echocardiography images of it.