Kawasaki disease (KD) is an acute vasculitis of childhood and is the leading cause of acquired heart disease in children in developed countries. Failure to quickly diagnose and treat patients with KD can result in severe cardiac sequelae, especially coronary artery aneurysms (CAAs). Patients with a prior diagnosis of KD who require general anesthesia (GA) may present unique challenges depending on the severity of any cardiovascular sequelae. This case report describes the perioperative management of a 5-year-old male patient previously diagnosed with incomplete KD approximately 1 year before presenting to Stony Brook University Hospital for full mouth dental rehabilitation under GA. Most uniquely, the patient was at high risk for coronary artery thrombosis due to a giant CAA of his right coronary artery and a small CAA of his left anterior descending artery. The discussion also includes the implications of dental treatment under GA for patients with a history of KD. © 2021 by the American Dental Society of Anesthesiology.