RESUMEN
Twelve-year-old patient, with congestive heart failure and mild, caused by Ebstein's anomaly with interventricular septal defect, was studied and submitted to surgical correction which consisted in covering the interventricular defect with a path of bovine pericardium and substituting for the tricuspid valve with a biological prosthesis (porcine). After surgical correction, the patient was submitted to strict clinical and laboratorial (echocardiogram and cardiac catheterization) evaluation which presented excellent adjustment of the cardiac antriums, with improvement of myocardial function index. The problems related to the diagnosis of the association of the Ebstein's anomaly with interventricular septal defect can be solved with a well done semiology, helped by accurate laboratorial procedures like echodopplercardiogram and cardiac catheterization. The total surgical correction can be achieved with results and, with this fact, change the natural development of the disease.