Most maternal cardiac disease in Western societies is now congenital in origin. This relates to the significant improvements in congenital cardiac surgery during the last years. Some patients will present for the first time in pregnancy with symptoms and learn that they have congenital heart disease. So all patients should have a detailed evaluation and appropriate counseling before pregnancy. Ebstein anomaly is a rare and complex congenital heart disorder occurring in 1 per 200 000 live births and, first described by a German physician Wilhelm Ebstein in 1866 in a report titled, “Concerning a very rare case of insufficiency of the tricuspid valve caused by a congenital malformation. This anomaly accounts for 0.5%–0.7% of cases of congenital heart disease. The average life duration of patients with Ebstein's anomaly is 25–30 years. The malformation consists of apical displacement of the tricuspid valve with resultant regurgitation and enlargement of the right heart chambers, resulting in arrhythmias and heart failure. The common cardiac anomalies associated with the condition are atrial septal defect (ASD) in 90% of patients, anatomic or functional tricuspid atresia in 30%, Wolff-Parkinson-White (WPW) syndrome in 15% and less commonly ventricular septal defect (VSD), pulmonic stenosis or atresia and mitral valve prolapse. Survival into adulthood is common and patients present with cyanosis, dyspnea and palpitations.