Abstract The association of complete atrioventricular canal with transposition of the great arteries is rare, with a prevalence of less than 3–5%. We present an 18-month-old patient with a complete atrioventricular canal, side-by-side transposition of the great arteries, and anomalous coronary anatomy, managed initially with pulmonary banding and then by arterial switch with complete atrioventricular canal repair at early infancy.