To investigate the clinical features, management, and outcomes of childhood Takayasu arteritis(c-TA) initially presenting with hypertension. This study retrospectively reviewed medical charts of 96 inpatient c-TA cases from 2002/01 to 2016/12, with 5 additional patients being prospectively recruited from 2017/01 to 2017/12. Data were compared between c-TA groups initially presenting with and without hypertension. Blood pressure(BP) control, event-free survival, and associated risk factors were assessed by logistic regression, Kaplan-Meier survival curve, and COX regression models. The hypertensive cohort(n=71, 28.2% males) as compared with non-hypertensive cohort had significantly fewer active diseases; fewer episodes of claudication, syncope, blurred vision, and myocardial ischemia; and fewer systemic symptoms(p<0.05). The hypertensive group presented with more localized abdominal lesions(OR=14.4, p=0.001) and limited supra-diaphragmatic arterial involvement. Renovascular disease(p=0.001) and revascularization (p=0.006) were associated with hypertension. At the 3-year follow-up, 53% of hypertensive patients achieved BP control and 39% experienced events including vascular complications, flares, or death. The 1-year, 3-year, 5-year, and 10-year event-free survival were 78.7%(95% CI, 65.7-87.2), 63.0%(95% CI, 48.1-74.7), 48.9%(95% CI, 32.0-63.8), and 31.6%(95% CI, 13.8-51.2) higher than in non-hypertensive group(p=0.014). Heart failure, stroke, and BMI <18.5 kg/m2 were prognostic factors for events. Intervention and baseline systolic BP were independent factors for BP control(p<0.05). Majority of c-TA has hypertension, presenting with a more quiescent disease without typical systemic and/or ischemia symptoms, more localized abdominal lesions, higher proportion of revascularizations and better event-free survival. Three-year BP control is over 50%. Intervention particularly on renal artery is beneficial for BP control and decreased events. © American Journal of Hypertension, Ltd 2019. All rights reserved. For Permissions, please email: journals.permissions@oup.com.