An anomalous origin of the left coronary artery from the pulmonary artery (Bland-White-Garland syndrome) was diagnosed in a 31-year-old woman complaining of angina at 10 weeks of gestation. After termination of pregnancy, the patient underwent surgical repair with ligation of the left coronary artery at the ostium, and a single bypass graft with a left internal thoracic artery to the left anterior descending coronary artery. Angiography, performed at 6 months of follow-up, showed stenosis of the distal anastomosis of the graft that was treated with angioplasty and deployment of a paclitaxel-eluting stent. After 9 months the patient was symptom-free and angiography excluded in-stent restenosis. Experience with the surgical repair of the anomaly is limited by the rarity of this condition; in particular, it has never been reported as a first diagnosis during pregnancy. In the discussion, we analyze the efficacy and limits of the different current strategies for the surgical repair of this rare, but potentially life-threatening, congenital coronary anomaly.