Juxtaposition of the atrial appendages (JAA) is an uncommon anomaly of the heart that is frequently associated with other cardiac abnormalities, such as transposition of the great arteries and tricuspid atresia. We encountered 7 patients with JAA from July 1984 to July 1989 at National Taiwan University Hospital. The JAA was right-sided in 1 patient and left-sided in 6. The patients' ages ranged from 10 months to 12 years. The cardiac anomalies in the patient with right JAA were double-outlet right ventricle and ventricular septal defect, which was repaired with a patch. Of the 6 patients with left JAA, 5 had transposition of the great arteries, and in 3 of them obstructive tricuspid lesions (tricuspid stenosis in 1, tricuspid atresia in 2) were noted. Three patients with left JAA and normal tricuspid valve underwent total correction (one Senning operation and two Rastelli operations). The other 3 patients with tricuspid obstruction underwent total cavopulmonary connection (2) and Fontan operation (1). We concluded that, first, left JAA was more frequently seen and associated with more morbid anatomy than right JAA. Second, if left JAA is discovered in planning biventricular repair for transposition of the great arteries, the tricuspid valve should be inspected carefully to rule out tricuspid obstruction.