BackgroundMoyamoya disease (MMD) management during pregnancy poses a challenge to health care providers, and recommendations are outdated, vague, and controversial. We conducted a systematic review to investigate and present the available evidence.MethodWe searched five online databases and bibliographies of relevant published original studies to identify case reports, case series, cohort studies, and reviews reporting on patients diagnosed with MMD before, during, or shortly after pregnancy. We report and analyze the respective data.ResultsFifty-four relevant articles were identified. In the group of patients with MMD diagnosed prior to pregnancy, 68.7% had previously undergone bypass surgery, 64.5% delivered via cesarean section, 95.2% of mothers had good outcomes, and no bad fetal outcomes were reported. In patients first diagnosed with MMD due to a cerebrovascular accident during pregnancy, the mean gestational age on symptom onset was 28.7 weeks and 69.5% presented with cerebral hemorrhage. In this group, 57.2% received neurosurgical operative management, and 80% underwent cesarean section with 13.6% maternal mortality and 23.5% fetal demise. In patients diagnosed with MMD immediately postpartum, 46.6% suffered a cerebrovascular event within 3 days of delivery, 78.3% of which were ischemic. Only 15.3% underwent surgical hematoma evacuation and one patient (9%) expired.ConclusionsMMD may coincide with pregnancy, but there is paucity of high-quality data. It appears that MMD is not a contraindication to pregnancy, if blood pressure and ventilation are properly managed. There is no clear evidence that bypass surgery before pregnancy or cesarean mode of delivery improve outcomes.