Ebstein's anomaly is a rare congenital heart disorder secondary to a malformation of the tricuspid valve and right ventricle. The heterogeneous spectrum of presentation of its structural anomalies and associated cardiomyopathy accounts for a widely varied clinical and hemodynamic manifestation of the pathology and its impact on timing of diagnosis and prognosis. Neonatal Ebstein's anomaly is characterized by reduced survival, while an average risk of mortality per year of 1% to 1.4% has been reported in infancy and adulthood. Medical management and a careful clinical and echocardiographic follow-up are advisable for all asymptomatic patients and those with minimal abnormalities. Surgical correction is recommended in presence of progressive dilation of the right atrium and right ventricle, development of right ventricular dysfunction, occurrence of supraventricular or ventricular arrhythmias or episodes of paradoxical embolization, reduced exercise capacity, or significant desaturation. Prosthetic valve replacement or repair of the tricuspid valve are both common strategies in the correction of tricuspid regurgitation. During the last three decades, important contributions in the development of repair techniques were driven from the experience of Danielson, Carpentier and da Silva, with satisfactory results in terms of safety and durability at long-term follow-up.