Aglossia-adactylia syndrome is a rare disorder characterized by aglossia and deformity of the limbs of variable degree. We managed a 2-day-old neonate with ileojejunal atresia with aglossia-adactylia syndrome. He was scheduled for the repair of the intestinal atresia, gastrostomy and palatal impression taking, under general anesthesia with sevoflurane supplemented with fentanyl. In this case we could achieve tracheal intubation by direct laryngoscopy with cricoid pressure applied, and could manage without any complications. But many of the patients with this syndrome are complicated with cleft palate and micrognathia, and we should be careful of the difficult airway at the induction of general anesthesia and aspiration during perioperative period.