Cervical aortic arch is rare with a prevalence of less than 1/10 000 live births (Pediatr Cardiol 10 (1989) 205); but may occur with other cardiovascular congenital abnormalities. We report pseudocoarctation of a left-sided cervical aortic arch with an aberrant right subclavian artery and aortic and mitral regurgitation, corrected by an anterior mediastinal shunt from ascending to descending aorta and aortic and mitral valve replacements, as a one-stage procedure.