Background <p>Aortic atresia with interrupted aortic arch is usually incompatible with life. The survival of a newborn is only possible with a coexistence of the other cardiovascular defects.</p> Case presentation <p>We report a newborn with aortic atresia, interrupted hypoplastic right aortic arch, bilateral arterial duct, (S, L,L) transposition of the great arteries and double inlet left ventricle. The right aortic arch was interrupted between the right and left subclavian artery. Blood supply to the very hypoplastic ascending aorta and coronary arteries was provided by right arterial duct, connecting the right pulmonary artery and the right subclavian artery. Through the left arterial duct the descending aorta and the left subclavian artery were perfused. On the 7th day of life, the child underwent selective bilateral pulmonary artery banding and 15 days later stent implantation in both ducti. Due to the tortuous course of the right duct the stent did not cover completely the duct and after prostaglandin PGE1 infusion discontinuation the child developed sign of cardiac ischemia. At the age of almost 8 weeks the child underwent a successful palliation by the modified Norwood procedure and 3 weeks after surgery, was discharged home gaining weight and clinically well.</p> Conclusions <p>We report a unique anatomy successfully palliated by modified Norwood procedure.</p>