Background. The combination of right aortic arch and coarctation of the aorta has seldom been reported. This rare abnormality occurs as an isolated lesion or in association with other congenital defects, such as mixed gonadal dysgenesis and Turner's syndrome. Methods. The medical records of 2 patients who underwent operation for right aortic arch and aortic coarctation in our institution were reviewed together with case reports in the literature. Various surgical options have been reported: synthetic grafts, subclavian flap, and end-to-end anastomosis. End-to-end anastomosis is our method of choice for coarctation of the aorta and for right aortic arch with coarctation, even though an excessive amount of dissection is needed. Results. Both patients from our institution are doing well, with no sign of recoarctation in either patient. Our experience and recent advances in the understanding of the anatomy of this lesion led us to develop an alternative simplified classification for right aortic arch, which is presented here. Conclusions. Right aortic arch and coarctation of the aorta is a rare morphologic combination. On the basis of our experience with repair of coarctation of the aorta and our review of the literature, we think that end- to-end anastomosis is the operative technique of choice with the best long- term results. Our simplified classification is easy to understand when dealing with right aortic arch.