Until recently, intracranial atheromatosis was a probably underdiagnosed clinicopathological entity that was rarely studied in depth. In the last years the advance and expansion in the use of non-invasive diagnostic tools have led intracranial atheromatosis to the front page among the most prevalent causes of stroke worldwide. Important efforts have been accomplished with the aim of identifying markers of poor outcome, which, besides the underlying mechanisms of cerebral ischemia in these patients, are the most important factors on which clinical and therapeutic decisions should be based. To date, the therapeutic armamentarium is scarce and far from optimun, regarding medical and endovascular measures. In this review we address the most important aspects of the natural history and cure treatment of intracranial atheromatosis.