We report a case of a 36 year old female with coronary artery obstructive disease(Left coronary osteal stenosis), who had been admitted due to severe headache and vomitting. In admission, she was diagnosed as moyamoya disease on cerebral angiogram. She had no history of hypertension, diabetes mellitus, hyperlipidemia, smoking. She had experienced angina for 2 years, and 1 year ago she ws diagnosed as bypass surgery with left main coronary artery angioplasty. In moyamoya disease, several portions of extracranial arteries have been found to be involved, but so far, only one case has been reported the coronary involvement on coronary angiogram in the world. And, there has not been a report about moyamoya disease combined with left main osteal lesion yet. This present case indicates that we need to exam for extracranial vascular system including the heart in moyamoya disease.