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To describe outcomes of HIV-infected pediatric patients with drug-resistant tuberculosis (DR TB).Demographic, clinical and laboratory data from charts of pediatric patients treated for DR TB during 2005–2008 were collected retrospectively from 5 multi-DR TB hospitals in South Africa. Data were summarized, and Pearson χ2 test or Fisher exact test was used to assess differences in variables of interest by HIV status. A time-to-event analysis was conducted using days from start of treatment to death. Variables of interest were first assessed using the Kaplan-Meier method. Cox proportional hazard models were fit to estimate crude and adjusted hazard ratios.Of 423 eligible participants, 398 (95%) had culture-confirmed DR TB and 238 (56%) were HIV infected. A total of 54% were underweight, 42% were male and median age was 10.7 years (interquartile range: 5.5–15.3). Of the 423 participants, 245 (58%) were successfully treated, 69 (16%) died, treatment failed in 3 (1%), 36 (9%) were lost to follow-up and 70 (17%) were still on treatment, transferred or had unknown outcomes. Time to death differed by HIV status (P = 0.008), sex (P < 0.001), year of tuberculosis diagnosis (P = 0.05) and weight status (P = 0.002). Over the 2-year risk period, the adjusted rate of death was 2-fold higher among participants with HIV compared with HIV-negative participants (adjusted hazard ratio = 2.28; 95% confidence interval: 1.11–4.68).Male, underweight and HIV-infected children with DR TB were more likely to experience death when compared with other children with DR TB within this study population.