A combination of hypoplasia of the left side of the heart, a hypoplastic aortic arch, a large patent ductus arteriosus, and valvular pulmonary stenosis is very rare. The severity of the valvular pulmonary stenosis in this setting is difficult to judge preoperatively. Here, we describe a newborn with borderline hypoplasia of the left side of the heart, a hypoplastic aortic arch, a large patent ductus arteriosus, and valvular pulmonary stenosis who underwent the Norwood procedure successfully. The purpose of this report is to share the perioperative course of the patient and our decision-making process that was not straightforward.