Cantrell syndrome is a congenital malformation with a pentalogy characterized by defects involving the abdominal wall, lower sternum, anterior diaphragm, and diaphragmatic pericardium, as well as congenital cardiac anomalies. We recently managed anesthesia in a patient with this syndrome and herein report our experience. The patient was a 14-day-old male neonate, who had been diagnosed with Cantrell syndrome, including ventricular septal defect, left ventricular diverticulum, abdominal wall defect, omphalocele, and sternal hypoplasia. Surgical interventions to close the ventricular septal defect, resect the left ventricular diverticulum, and close the omphalocele were scheduled. After cardiac surgery, the hernial contents were returned to their original compartment and, subsequently, an attempt was made to suture the abdominal wall. However, blood pressure fell markedly and the attempt was discontinued. The chest was left open postoperatively and the patient was transferred to the intensive care unit (ICU), during which time circulatory and respiratory management was very complex. Issues requiring particular attention in the management of anesthesia for patients with this syndrome include complications of diverse cardiac malformations, pulmonary hypertension, pulmonary hypoplasia, and respiratory and circulatory failure associated with increased intraabdominal pressure due to primary closure of the omphalocele. Accordingly, extreme caution must be taken to restore respiratory and circulatory control.