In 1866, Wilhelm Ebstein published a scholarly description of a tricuspid valve anomaly with dilation of the right atrium and patent foramen ovale that bears his name. However, his original report was almost overlooked. Despite a wide range of publications on the history of cardiac pathology and cardiac surgery, the international literature provides only scarce information regarding the personality of Wilhelm Ebstein and his original description of the anomaly that bears his name. In this article, we present biographical data of Wilhelm Ebstein and discuss how his original description of autopsy findings correlates with our current knowledge of this congenital disorder. It is the excellent correlation of Ebstein's pathologic findings with clinical notes of his colleague and Ebstein's hypotheses of the pathophysiology that made his publication a landmark in the description of a new entity. In addition, Ebstein's report provided a strong basis for the development of repair techniques for this rare anomaly 100 years later.