This study aimed to elucidate the clinical features, surgical treatment and long-term outcomes of adolescent patients with moyamoya disease. We reviewed consecutive patients with moyamoya vasculopathy who were admitted to our hospital from 2009 to 2015 to identify adolescent patients with moyamoya disease. Clinical features, surgical treatment and long-term outcomes were analyzed. Follow-up was performed by face-to-face or structured telephone interviews. Outcome measures were future stroke events. We performed univariate and multivariate time-to-event analyses to identify risk factors associated with future stroke events. A total of 95 adolescent patients with moyamoya disease (age at onset, 13.1 +/- 2.3 years) were included in this study. During follow-up, 12 patients (12.6%) had stroke events. We found that the patients who underwent direct/combined bypass had a significantly lower risk of future strokes [hazard ratio (HR), 0.16; 95% confidence interval (CI), 0.03-0.74; P = 0.019] compared to patients who underwent indirect bypass. Our results demonstrate that direct/combined bypass can be more effective in preventing future strokes than indirect bypass in adolescent patients with moyamoya disease.