Atherosclerotic disease of the intracranial arteries is responsible for at least 10% of ischaemic strokes worldwide. Symptomatic disease has been extensively studied in the past few years, using diagnostic methods including multi-slice computed tomography and high resolution magnetic resonance imaging. A literature search was performed using PubMed and OvidSP between 1984 and May 2013. Variations of the terms ‘intracranial atherosclerosis’ plus ‘ischemic stroke’, ‘plaque’, ‘morphology’, ‘imaging’ were used and a combination of them. The reference lists of identified articles were also consulted for additional references. Amongst symptomatic patients the prevalence of intracranial atherosclerotic disease is around 10%, depending on race ethnicity, and the diagnosis requires the presence of ≥50% stenosis in the territory of the symptomatic vessel in a patient with stroke or transient ischaemic attack. The prognosis of intracranial atherosclerotic disease related stroke is poor. Although risk factor control can lead to a better outcome of intracranial atherosclerotic disease related strokes, the significance of asymptomatic disease is still a matter of debate.