The rare case of a 58 years old male patient with a combination of Ebstein's disease and coarctation of the aorta is reported. The patient presented and died with manifestations of cardiac and respiratory failure. The post mortem examination revealed the combination of Ebstein's disease and coarctation of the aorta. The analysis of the clinical and paraclinical data points to an atypical course of the disease probably due to the opposite hemodynamic characteristics of the two congenital heart diseases.