Background: Cold-induced urticaria is an uncommon but well described phenomenon in which a spectrum of responses may result from exposure to a cold stimulus. Patients with cold-induced urticaria who require cold cardiopulmonary bypass are at risk for hypotensive episodes. Objective: To describe the case of a 69-year-old man with documented cold-induced urticaria who required aortic valve replacement and coronary artery bypass surgery. Methods: After receiving a prophylactic anti-inflammatory regimen, the patient underwent cold cardiopulmonary bypass. After systemic cooling to 32degreesC, cold blood cardioplegia was administered at 4degreesC to obtain initial cardiac standstill. Thirty minutes before anticipated rewarming, anti-inflammatory medications were again administered. After rewarming to 37degreesC for more than 33 minutes, he was successfully weaned from cardiopulmonary bypass without inotropic or pressor support and with normal pulmonary compliance. The prophylactic regimen was continued postoperatively. Results: The patient was extubated 11 hours after surgery, and with the exception of a brief, self-limited episode of atrial fibrillation, his course was uneventful. He experienced no urticaria, angioedema, or hypotension and was discharged home on the fourth postoperative day. Conclusions: Although it is likely that the need for cold cardiopulmonary bypass surgery in patients with cold-induced urticaria is uncommon, it is encouraging that such a regimen may allow for the successful completion of the surgery.