Congenitally corrected transposition of the great arteries (CCTGA) accounts for <1% of all cases of congenital heart defect, a pathological condition characterized by the existence of both ventriculoarterial and atrioventricular (AV) discordance in the heart. CCTGA is more commonly associated with type B interrupted aortic arch (IAA) than type A variant. This is a more intricate and unusual presentation than dextro-transposition of the major arteries with an IAA. Herein, we present a case of an extraordinarily rare congenital cardiac complex defect. CCTGA and type A IAA were found in a 34-week preterm infant weighing 2.4 kg at delivery. Antenatally, the fetal echocardiogram suggested CCTGA in the form of an apically displaced left AV valve ventricular septal defect and transposed great arteries. The pulmonary trunk appeared larger than the aorta with three abnormal vessels. This study emphasizes the usefulness of sequential imaging modalities including fetal echocardiography to determine the majority of the anatomic details.