Cold-induced urticaria is a chronic urticaria subtype which can result in a severe, life-threating, anaphylaxis type reaction to cold stimuli. While a rare condition, it has potentially significant implications in cardiac surgery, where a degree of hypothermia may often be used for neurological or myocardial protection. We present a case of the successful perioperative management of this condition for surgical aortic root replacement with a mechanical aortic valve conduit, pulmonary vein isolation and left atrial appendage clipping performed on cardiopulmonary bypass. Review of the limited number of previously published case reports, current non-perioperative management guidelines and an immunology specialist consult informed our perioperative plan. A 5-day oral course of high-dose second-generation H1-antihistamine was provided pre-operatively. High-dose oral H1-antihistamines were continued over the immediate perioperative period, in addition to a short course of corticosteroids. No H2-antihistaminergic agents or additional intraoperative intravenous H1-antihistamines were used. Intraoperatively, systemic cooling was limited to 32°C on cardiopulmonary bypass and active warming used at other times. No manifestations of cold-induced urticaria occurred perioperatively demonstrating this can be an effective treatment strategy.