Ebstein's anomaly is a rare malformation of the tricuspid valve that has anatomopathologic and clinical implications, accounting for < 1% of all cardiac malformations. Usually, the tricuspid valve has three leaflets and, if Ebstein's anomaly occurs, such leaflets are malformed: one-two leaflets are stuck to the wall of the heart so that they cannot move normally, furthermore other abnormalities can also coexist. Therefore, the awareness of the different anatomical, hemodynamic and clinical variables of Ebstein's anomaly is crucial to treat properly, surgically or not, such malformation. The present paper takes into account current natural history, diagnostic criteria and classification, echocardiographic features, and prognosis useful to the accurate recognition and management options of Ebstein's anomaly.