A 15-year-old girl with pseudoxanthoma elasticum and associated severe coronary artery disease underwent double coronary artery bypass using left internal mammary artery and saphenous vein. Preoperatively the patient had life-threatening syncope and positive treadmill test results. The postoperative course was uneventful, and the patient has been doing well for 3 years. Pseudoxanthoma elasticum is a rare disease that can cause premature coronary arterial obstructive disease in children and adolescents.