Coarctation of the aorta is any form of narrowing of the aorta; it is often a discrete narrowing of the aorta distal to the left subclavian artery, just past the point of insertion of the ductus arteriosus. The hallmark is upper extremity hypertension characterized by a differential systolic blood pressure gradient between the upper and lower extremities of at least 20 mm Hg. If left untreated the natural history involves development of accelerated coronary artery disease, stroke, heart failure, and/or aortic dissection. In neonates the treatment of choice is a surgical repair but in older children the decision to utilize a surgical versus an interventional cardiology approach in the catheterization laboratory is more complex. Craniosynostosis is a condition in which one or more of the sutures in an infant’s skull fuse prematurely and alter the growth pattern of the skull; it is surgically repaired, often during infancy. This chapter discusses the perioperative challenges of caring for an infant with residual coarctation of the aorta undergoing craniosynostosis repair.