A 43-year-old woman was diagnosed with moyamoya disease (MMD) and underwent right-side bypass surgery. After surgery, previous symptoms disappeared. One month later, transient right hemiparetic attacks and motor dysphasia developed. Angiography revealed progressive severe stenosis of left supraclinoid segment of internal carotid artery. Angioplasty using a drug-eluting stent (DES) was performed. For 18 months, she presented no ischemic symptom and no instent stenosis was observed in follow-up angiography. This is the first case report about effect of DES use for MMD. Considering that intimal hyperplasia is a pathophysiology of stenosis, DES may have a role in reducing progression of stenosis in selected moyamoya patients.