Background. Moyamoya disease is a cerebrovascular disease that is characterized by bilateral chronic and progressive stenosis or occlusion of the arteries around the circle of Willis with development of collateral circulation, of unknown etiology. It has a high incid ence in Japan and Asian population, with fewer cases described in Europe. Similar angiographic findings can be seen in patients with other medical conditions that are described as Moyamoya syndrome. Main clinical features include transient ischemic attacks , ischemic strokes, and hemorrhagic strokes. Case report. We describe a 38 year old female patient that presented with an intracerebral hemorrhage with a typical location for hypertensive bleeds. She had no vascular risk factors, but a high normal blood p ressure (140/90 mmHg), and elevated ESR. A magnetic resonance angiography showed occlusion of internal carotid artery with development of collateral cerebral circulation on the side of the bleeding. Unilateral affection and elevated ESR were more character istic for a moyamoya syndrome within a systemic disease. Conclusions. Despite a typical hypertensive location of the bleeding, vascular imaging is warranted in all patients with intracerebral bleedings to evaluate for atypical etiologies. Our case represe nts a patient that might benefit from revascularization surgery in the context of multifactorial risk factor control.