Bicuspid aortic valve (BAV) is the most common congenital cardiac defect, commonly associated with valve dysfunction and coarctation of aorta. Rarely, BAV can be associated with abnormalities of the coronary arteries, the most common of which are ectopic coronary origins. In this report, we present a case of a 2-year-old child with BAV, without coexisting supravalvular aortic stenosis, who was found to have a left main coronary ostio-proximal stenosis, leading to ischemic cardiomyopathy and congestive heart failure.