A 1-month-old girl was referred at our Institution with a history of heart failure and cyanosis. We established diagnosis of multiple congenital heart disease with criss-crossed atrioventricular connections but concordant. This type of anomaly has a variety of presentations of high diagnostic complexity. The multiple lesions included double outlet of the right ventricle, right subaortic stenosis, ventricular septal defect, coarctation of the aorta with hypoplasia of the arch, and persistent patency of the arterial duct, established through echocardiography, angiocardiography and magnetic resonance. These findings prompted palliative surgery and correction of the aortic arch.