Carotid duplex ultrasound is the standard of care for the initial diagnosis of carotid artery bifurcation diseases. But in difficult examinations, carotid abnormalities are commonly encountered and may represent a diagnostic challenge in patients with clinical symptoms as well as in the follow up after carotid endarterectomy or carotid artery stenting. Contrast enhanced ultrasound (CEUS) with low mechanical index (low MI) is a promising new method in the diagnosis and follow up of pathological carotid diseases. Unlike most contrast agents used for magnetic resonance imaging or computed tomography, the microbubbles used in CEUS with SonoVue(®) remain within the vascular space and hence can be used to study vascular disease. In addition to improving current carotid structural scans, CEUS has potential to improve or add extra information on carotid arterial diseases. This review describes the current carotid duplex ultrasound examination and compares the pathological findings with CEUS.