Intracranial mycotic aneurysm (IMA) is rare, but life-threatening cerebrovascular lesion, which arises from a variety of primary infection foci such as infective endocarditis, bacterial meningitis, cavernous sinus thrombophlebitis, etc. The diagnosis of IMA usually depends on the documentation of an intracranial aneurysm by vascular imaging in the presence of primary infection foci. As the gold standard for detecting IMA, high-tech computed tomography will eventually replace cerebral angiography. Because of the lack of prospective cohorts and randomized controlled trials, there is no widely accepted treatment guideline for IMA. With recent advances in surgical technique and the introduction of endovascular therapy, however, clinical outcome of patients with IMA tends to improve. This review will provide the state-of-the-art knowledge on clinical aspect and treatment strategy of IMA.