Stroke during pregnancy or the puerperium is an extremely rare yet serious cause of perinatal morbidity and mortality. Moyamoya disease, a cerebrovascular occlusive pathology with a female predominance, may become symptomatic for the first time in association with pregnancy. A 36-year-old woman with postpartum pre-eclampsia suddenly developed hemiparesis in the left arm with dysarthria after the initiation of antihypertensive measures. Cranial magnetic resonance imaging and angiography revealed acute ischemic lesions in the right hemisphere along with the steno-occlusive lesions of bilateral terminal portions of the internal carotid artery, indicating Moyamoya disease. With anti-platelet medication, the patient recovered gradually and was diagnosed as having Moyamoya disease using conventional angiography 3 months postpartum. In this case, the fluctuations in blood pressure in association with pre-eclampsia appear to have exacerbated the clinical symptoms of Moyamoya disease. As such, Moyamoya disease should be considered as an underlying disease of ischemic stroke associated with pregnancy.