A stroke due to underlying intracranial large artery occlusion, which is atherosclerotic in nature, is known as intracranial atherosclerotic disease (ICAD). It is important to recognize that ischaemic stroke due to ICAD differs from extracranial disease and other stroke aetiologies and requires a nuanced approach. It is a significant cause of stroke worldwide, and severe symptomatic ICAD can present challenges from a therapeutic standpoint, including recurrent ischaemic stroke despite optimal management. Furthermore, exploring the underlying pathophysiological mechanisms responsible for the disease may be necessary while considering treatment options. This narrative review aims to provide an all-encompassing overview of this disease. Epidemiology and clinical pathophysiology will be explored in detail. The findings of large clinical trials will serve as a guide to finding the most optimized management strategies. Another critical question that arises is the treatment of acute ischaemic stroke due to large vessel occlusion with underlying intracranial atherosclerosis, is the treatment and clinical diagnosis the same as for other aetiologies of stroke (i.e. extracranial disease and nonvalvular atrial fibrillation)? Consequently, secondary prevention of patients with ischaemic stroke or transient ischaemic attack will be divided into medical therapy, risk factor control, and endovascular and surgical treatment options.