Moyamoya disease (MMD) is a specific chronic cerebrovascular occlusive disease which is characterized by progressive occlusion of the vasculature, predominantly in the brain. Many branches of collateral circulation develop and give rise to the angiographic appearance of a ”puff of smoke” that is pathogonomic of this disease and is also the origin of its name in Japanese. In addition to ischemic insults to the brain, various types of complicated intracranial hemorrhaging often occur. Herein we reported a case who presented with intracranial hemorrhage. The associated literature was reviewed MMD is a rare disease in Taiwan in comparison to Japan. However, this disease must be considered in the differential diagnosis treating a young patient with infarction stroke or non-traumatic intracranial hemorrhage.