Coarctation of aorta associated with severe hypoplastic aortic arch and ductus arteriosus dependent, often combined with complex cardiac malformations, should be looked upon as bordering of hypoplastic left heart syndrome. The crucial and first objective is the adequate reconstruction of aortic arch, continuing with the repair of cardiac malformations. The surgical treatment making resection of the coarctation segment combined with left carotid flap plasty is a surgical alternative, useful in patients with this complex anatomic variant.