The plain roentgenographic and angiocardiographic findings and pertinent laboratory data of a case ofEbstein's anomaly were presented with a brief review of literatures. A 39-year-old woman was admitted to St.Mary's Hospital for the evaluation of cardiac disease. The patient complained of exertional dyspnea, generalizededema, abdominal distention, and amenorrhea for 11 years. Physical exmination revealed malar flush, puffy face,and engorgement of neck vein. heart sound was distant on auscultation, but there was no murmur. EKG revealedarrhythmia, atypical RBBB, right axis deviation, frequent premature atrial contracture and low voltage.Phonocardiogram showed splitting of the 2nd and 4th heart sounds. The frontal view of the chest demonstratedmarked cardiomegaly with typical " box-like" appearance. The peripheral pulmonary vasculatures were markedlyreduced. The angiocardiogram showed atrium was enormously enlarged. The auricularized right ventricle, tremendousdilatation of the functioning right ventricle and a small sinus potion were characteristic. Angiocardiographyconfirmed the diagnosis of Ebstein's anomaly.