Uhl’s anomaly (a.k.a. “Parchment Heart”) is a rare congenital heart condition where the myocytes are absent in the right ventricle which results in right ventricular failure and arrhythmia during infancy. The right ventricular wall is very thin and hypocontractile. The endocardium essentially connects directly to the epicardium without the muscle layer in between. The treatment is surgical repair frequently with a bidirectional Glenn shunt which is performed by closing the tricuspid valve and creating an atrial septal defect, effectively excluding the right ventricle. It is important to note that Uhl’s anomaly is different than arrhythmogenic right ventricular dysplasia (ARVD).