Introduction Ebstein anomaly is a congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialization of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. Wilhelm Ebstein first described a patient with cardiac defects typical of Ebstein anomaly in 1866. In 1927, Alfred Arnstein suggested the name Ebstein's anomaly for these defects. In 1937, Yates and Shapiro described the first case of the anomaly with associated radiographic and electrocardiographic data Definition Ebstein anomaly is a congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialization of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. Epidemiology Ebstein anomaly probably accounts for 0.5% of cases of congenital heart diseases. Its true prevalence is unknown because mild forms frequently are undiagnosed. With the wide application of echocardiography, more cases are being diagnosed. Race-, sex-, and age-related demographics Ebstein anomaly is more common in children of white females. However, no specific sex predominance exists. Ebstein anomaly can present at various stages of life, as follows: • Fetal life: Ebstein anomaly is usually diagnosed incidentally by echocardiography. • Neonatal life and infancy: Ebstein anomaly presents with cyanosis and/or severe heart failure; typically, symptoms present in infancy improve as pulmonary vascular resistance decreases.