The surgical repair of truncus arteriosus associated with an interrupted aortic arch (TAC-IAA) requires performing two major procedures at the same time. Due to the small number of patients, there is nearly no surgical learning curve. The surgical technique has greatly improved since the introduction of a homograft patch enlargement of the small ascending aorta. The association with a severe truncal regurgitation is a major risk factor as well as the presence of preoperative multiple organs failure. The series published by single centers are ≪10 patients, which make statistical analysis troublesome. The mortality varies from 0% to 50%. The multicentric study published in 2006 by the Congenital Heart Surgeons Society (CHSS) reports a 68% mortality (34/50). Nevertheless, the results can be excellent in experienced centers using a modern one stage surgical technique, undertaken in the first week of life.