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Objectives: To describe the epidemiology and factors associated with pediatric central nervous system (CNS) tuberculosis (TB) in California from 1993 to 2011., Methods: We analyzed California TB registry data for persons aged ≤18 years, comparing CNS TB cases versus non-CNS TB cases reported from 1993 to 2011. Factors associated with CNS TB and TB deaths were identified by using multivariate logistic regression., Results: A total of 200 CNS TB cases were reported. Compared with non-CNS TB case patients, CNS TB case patients were more likely to be aged <5 years (72.0% vs 43.6%; odds ratio [OR]: 3.8 [95% confidence interval (CI): 2.4-5.9]), US-born (82.0% vs 58.2%; OR: 3.3 [CI: 2.3-4.7]), and Hispanic (75.0% vs 63.2%; OR: 1.7 [CI: 1.3-2.4]). Among US-born CNS TB case patients (during 2010-2011), 76.5% had a foreign-born parent. Tuberculin skin test results were negative in 38.2% of 170 CNS TB cases tested. In multivariate analysis, age <5 years (adjusted odds ratio [aOR]: 3.3 [CI: 2.0-5.4]), US birth (aOR: 1.8 [CI 1.2-2.7]), and Hispanic ethnicity (aOR: 1.5 [CI: 1.1-2.1]) were associated with an increased risk of developing CNS TB. For deaths, CNS TB (aOR: 3.8 [CI: 1.4-9.9]) and culture positivity (aOR: 6.2 [CI: 2.2-17.3]) were associated with increased risk of death, whereas tuberculin skin test positivity (aOR: 0.1 [CI: 0.04-0.2]) was associated with decreased risk., Conclusions: Subsets of children are at increased risk for CNS TB in California and may benefit from additional prevention efforts., (Copyright © 2015 by the American Academy of Pediatrics.)