A conventional grey scale, Doppler sonography and color-coded Doppler ultrasound examination should be the first step in the diagnosis of the vessels supplying the brain and have a major impact in diagnostic imaging. This ultrasound examination allows the simultaneous acquisition of morphologic and hemodynamic information with high spatial and temporal resolution. Most of the pathological changes of the extracranial arteries can thus be reliably detected. Contrast-enhanced ultrasound (CEUS) is a promising new non-invasive method for the diagnosis and follow-up of complex carotid abnormalities. In addition to improving current carotid structural scans, CEUS has the potential to improve or provide additional information on carotid arterial diseases, such as the reliable differentiation between internal carotid artery (ICA) occlusion and pseudo-occlusion, improved visualization of in-stent stenosis, extracranial ICA aneurysms and carotid dissection.In this article the contribution of color-coded duplex ultrasound and CEUS in assessing various pathologies of the carotid artery will be addressed.