We are reporting a case of Ebstein's anomaly of the tricuspid valve and another case of a congenital double tricuspid lesion, both associated with pulmonary valve stenosis and with similar hemodynamics and physiopathology. Given that there are few reported cases in the literature, we analyzed the clinical and electrocardiographic findings of both. The utility of the echocardiogram is discussed in the diagnosis of such abnormalities and the angiographic and hemodynamic data commented upon. It is emphasized that right ventricular hypertension (105 mm Hg, and 98 mmHg, respectively) modifies the cardiopathic hemodynamics and its natural history, that was favorably modified by isolated corrective surgery of the pulmonary obstruction. The postoperatory transvalvular gradients were measured at 27 and 11 mm Hg, respectively. After having revised the pertinent bibliographic literature, we commented upon the factors that give such a high mortality rate in Ebstein's surgery and the congenital abnormalities which are most frequently associated.