A 73-year-old man presented to our department for ischemic alterations appeared during a stress electrocardiogram. The diagnostic process including coronary angiography, cardiac computed tomography and echocardiogram revealed an anomalous origin of the left main coronary artery from the commissural region between the left and the non-coronary Valsalva sinus associated with non-obstructive accessory mitral valve tissue. Although these malformations are relatively common the association is not described in literature to date.