Intracranial atherosclerosis is more prevalent in Asian patients, whereas extracranial atherosclerosis is more common in individuals from western countries. The reasons for this discrepancy remain unknown. We reviewed the relevant literature and discussed the currently available information. Although the study population, diagnostic modality, and risk factor definitions differ between studies, hypercholesterolemia is more correlated with extracranial atherosclerosis than intracranial atherosclerosis. The difference in hypercholesterolemia prevalence is one of the main reasons for racial differences. Intracranial arteries contain higher antioxidant level than extracranial arteries and may be more vulnerable to risk factors for antioxidant depletion (e.g., metabolic syndrome and diabetes mellitus). Intracranial arteries may be vulnerable to factors associated with hemodynamic stress (e.g., advanced, salt-retaining hypertension and arterial tortuosity) because of a smaller diameter, thinner media and adventitia, and fewer elastic medial fibers than extracranial arteries. Additionally, non-atherosclerotic arterial diseases (e.g., moyamoya disease) that commonly occur in the intracranial arteries of East Asians may contaminate the reports of intracranial atherosclerosis cases. Genes, including RNF 213 or those associated with high salt sensitivity, may also explain racial differences in atherosclerotic location. To understand racial differences, further well-designed studies on various risk and genetic factors should be performed in patients with cerebral atherosclerosis. Additionally, improvements in diagnostic accuracy via advancements in imaging technologies and increased genetic data will aid in the differentiation of atherosclerosis from non-atherosclerotic intracranial diseases.