Ebstein’s anomaly is a rare heart defect affecting the tricuspid valve and right ventricle. The tricuspid valve is dysplastic, with downward displacement of the septal and posterior leaflets inferiorly into the right ventricle. While the mildest forms of Ebstein’s anomaly include minimal tricuspid regurgitation and tricuspid valve displacement, more severely affected patients will have greater tricuspid valve displacement with resultant loss of right ventricular volume and right ventricular dysfunction. An atrial level communication, either an atrial septal defect or patent foramen ovale, also exists in nearly all patients. In more severely affected patients right-to-left shunting at the atrial level results in varying degrees of cyanosis. This chapter discusses the considerations involved in the perioperative care and management of a patient with mild right ventricular dysfunction and tricuspid regurgitation undergoing urgent noncardiac surgery.