The most common abnormal relationship between the great arteries and ventricle is transposition of great arteries, among which complete D-transposition is a common and lethal malformation. Without appropriate treatment, the infant born with transposition of the great arteries rarely survives the first year of life. In contrast to the grim hopelessness of only a few years ago, modern aggressive palliative and corrective surgical interventions can provide such infants with considerable hope for adolescent and adult life. Up until this time, intraatrial baffle technique for re-direction of venous return, which was proposed by Mustard originally, has been successfully applied to infants and children in many foreign clinics with decreasing trend of the operative risk. In this report, we present one case of a 4 year-old girl having complete D-transposition of the great arteries with atrial septal defect, and reviewed the relevant literatures.[KTCS 1979;3:197-206]