The blue toe syndrome is characterized by tissue ischemia secondary to cholesterol crystal or atherothrombotic embolization leading to occlusion of small vessels. Embolization occurs typically from anulcerated atherosclerotic plaque located in the aorto-iliac-femoral arterial system. Clinical presentation can range from a cyanotic toe to a diffuse multiorgan systemic disease that can mimic other systemic illness. Mortality can be higher than 70% depending on the scope of the illness. Embolization can occur spontaneously or from a variety of insults such as invasive vascular procedures, anticoagulation, or thrombolytic therapy. Angiography, duplex ultrasonography, computerized tomographic scanning, and magnetic resonance imaging have been used to image the offending lesions, with angiography considered the “gold standard” despite its inherent risks. Recently, transesophageal echocardiography has been shown to be a helpful tool in imaging the thoracic aorta and delineating in great detail the anatomy of the aortic atheroma. At present, surgery remains the most viable treatment option. However, we look to the future for large randomized trials to help predict embolization and thus the propermedical therapy.