Interrupted right sided aortic arch is a very rare anomaly, and to our knowledge only one such case in which the patient underwent complete repair has been recorded in the literature. Twelve additional cases were found on autopsy or underwent palliative surgery. We present the case of a 25-day-old infant with an interrupted right sided aortic arch. The anomaly was repaired by a one-stage surgical approach, and the patient discharged from the hospital. Surgical technique considerations are discussed.