We report on a case where craniosynostosis surgery for a left-sided coronal synostosis was performed successfully on an 11-month old infant with a hypoplastic left ventricle with a dysplastic mitral valve, double outlet right ventricle, transposition of the great arteries, atrial septal defect, multiple ventricular septal defects, and surgically applied pulmonary banding. Craniosynostosis surgery is considered high-risk surgery, because of possible sudden and extensive blood loss, and is usually performed in cardiopulmonary healthy children. Children with congenital heart disease undergoing noncardiac surgery have an increased risk of perioperative morbidity and cardiac arrest. Patients with hypoplastic left heart syndrome are a high-risk population when undergoing noncardiac surgery, in all stages of palliation. This infant would be undergoing a partial cavo-pulmonary connection (PCPC) within a few months. With a PCPC, drainage of cranial vessels is dependent on passive flow via the superior caval vein directly into the pulmonary artery. Consequently, this could lead to an increased blood loss during craniosynostosis surgery. Therefore, it was decided to perform the craniosynostosis surgery first, before establishing a PCPC. When a child with CHD presents for high-risk noncardiac surgery, future cardiac procedures and physiology also have to be taken into account. A multidisciplinary approach, involving pediatric cardiologists and pediatric anesthesiologists, is essential in making this decision.