This chapter presents a case study of a 7-year-old female with a history of hypoplastic left heart syndrome (HLHS) who was status post Fontan completion operation 3 years ago and who presented for resection of a right temporal lobe mass. The primary concerns of the anesthesiology team included the avoidance of increased pulmonary vascular resistance (PVR), the maintenance of adequate systemic oxygenation, the avoidance of increased intracranial pressure, and the maintenance of adequate cerebral perfusion pressure. When managing a congenital heart disease (CHD) patient for noncardiac surgery it is imperative to address the specific anatomy and physiology following any palliative procedures. The history and physical examination is essential in determining the presence of congestive heart failure. Postoperative pain management can include carefully titrated opioids such as fentanyl, hydromorphone, or morphine with special care to avoid respiratory compromise that can result in hypercarbia, hypoxia, atelectasis, and increased PVR.