BACKGROUND: Despite being the most common presentation in children with moyamoya, transient ischemic attack (TIA) in children has rarely been described. The aim of this study is to describe the clinical characteristics of TIAs in children with moyamoya and explore the risk factors for stroke after TIA. METHODS: We reviewed 696 consecutive patients with moyamoya vasculopathy (155 pediatric patients and 541 adults) admitted to our hospital from 2009 to 2015 to identify pediatric patients with moyamoya with an initial presentation of TIA. We defined recurrent TIAs that involve more types of symptoms or symptom extensions as symptom progression. The risk factors for subsequent stroke were analyzed using time-to-event analyses. RESULTS: We identified 60 pediatric patients with moyamoya who had presented with TIA (initial presentation age, 10.0 +/- 3.5 years). Motor weakness (n = 51 [85%]) was the most common initial presentation. During follow-up, 55 patients (91.7%) had recurrent TIAs and 14 (23.3%) had subsequent strokes. We identified female gender (hazard ratio, 5.08; 95% confidence interval, 1.40-18.47; P = 0.01), Suzuki grade greater than 3 (hazard ratio, 4.01; 95% confidence interval, 1.16-13.82; P = 0.03), and symptom progression (hazard ratio, 5.31; 95% confidence interval, 1.6517.14; P = 0.01) as independent predictors of future stroke events. CONCLUSIONS: Transient ischemic attacks have a relatively high recurrence rate in children with moyamoya and are associated with subsequent stroke. We identified the female sex, Suzuki grade greater than 3, and symptom progression as independent predictors of future strokes.