Moyamoya disease is a progressive occlusive disease of the circle of Willis with prominent collateral arterial formation. We report on a 12-year-old girl with moyamoya disease presenting with transient ischemic attacks (TIAs). Surgical indirect revascularization was performed. The patient did not suffer further TIAs at 12 month follow-up. Pre and postoperative cerebral perfusion were studied in quantitative single photon emission computerized tomography (SPECT) and CT perfusion imaging. CT perfusion imaging demonstrated postoperative increased cerebral blood flow as well as SPECT before and after revascularization. Furthermore, the area of decreased vascular reserve in SPECT with acetazolamide corresponded with areas of increased cerebral blood volume in CT perfusion imaging. CT perfusion imaging was equivalent to SPECT in accuracy, and superior in spatial resolution. CT perfusion imaging is likely to become more widely available as an easy-to-perform technique for assessing cerebral perfusion in a patient with moyamoya disease.