Bart's syndrome, first described by Bart in 1966, consists of congenital localized absence of skin, congenital epidermolysis bullosa, and associated nail abnormalities. A newborn infant with Bart's syndrome is reported since it is a very rare condition, especially when associated with pyloric and concomitant choanal atresia. To the best of our knowledge, this is the first report presenting a case of Bart's syndrome associated with choanal atresia.