Ebstein's anomaly is a rare congenital heart defect characterized by apical displacement of the tricuspid valve and reduced volume of the true functional right ventricle. In its most severe form, the disease presents as right heart failure and cyanosis in the neonate. Although many of these neonates can be managed conservatively, surgical intervention may become necessary in the sickest of them. Surgical intervention in this subgroup can be challenging and requires meticulous appreciation of the anatomy and physiology on a case to case basis. Multicenter studies are required to define a uniform algorithm and approach towards these patients.