Cantrell syndrome is a rare syndrome associated with varying degrees of midline wall defects and congenital cardiac anomalies. In this article, we report a six-year-old girl with Cantrell syndrome associated with single ventricle, pulmonary stenosis, ventricular diverticulum, bilateral superior vena cava, lower sternal, and supra-umbilical abdominal anterior wall defect. The patient, who had no previous intervention despite severe cyanosis (SpO(2): 65 to 70%) due to the parent decision, underwent operation. Bilateral Glenn procedure, resection of the ventricular diverticulum, and anterior abdominal wall repair were performed in collaboration with pediatric surgery. The patient was discharged uneventfully with a SpO(2) of 80 to 85%. No problem was observed in the postoperative sixth month visit.