Truncus arteriosus (TA) is a congenital heart defect often diagnosed in neonatal period; it represents 0.7% of all congenital heart lesions. The pulmonary arteries originate generally above the coronary ostium. Aorto-pulmonary and interventricular defects are believed to represent an abnormality of conotruncal septation. TA is classified into four types, according to Van Praagh and Colette Edwards. Some of congenital heart defects may be associated with TA, such as aortic interruption. In literature, TA with a double aortic arch is observed as a very rare condition. We present here a newborn diagnosed with a combination of TA type 1 and double aortic arch.