Three patients with an anomalous main coronary artery coursing between the great vessels are presented with a review of the literature. Their surgical treatment by coronary artery bypass grafting with use of the ipsilateral internal mammary artery is described with angiographic follow-up. Young patients with these anomalies, whether they are asymptomatic or have syncope, are at high risk for sudden death. Older patients with angina appear to be adequately risk stratified by thallium stress tests. For patients requiring surgical intervention, aortoplasty and coronary artery bypass grafting have both resulted in relief of symptoms, but the follow-up is limited. We suggest that coronary artery bypass grafting with an ipsilateral internal mammary artery graft to the anomalous vessel is the procedure most likely to relieve ischemia and achieve good long-term results for both symptomatic relief and survival.