Single-center studies have reported that HIV-associated nephropathy (HIVAN) can occur in children and may have a clinical course and prognosis similar to that of adults. However, the prevalence and survival has not been reported for a national sample of children with HIVAN and end-stage renal disease (ESRD) on dialysis in the United States. We utilized the United States Renal Data System (USRDS) database to determine the prevalence, demographic information, and survival of children with HIVAN and ESRD in the United States. The Kaplan-Meier method was used to estimate survival of children with HIVAN and the log-rank test was used to compare their survival with children with focal segmental glomerulosclerosis (FSGS) and adults with HIVAN. Cox regression analysis was used to model adjusted hazard ratios (AHR) with HIVAN as a cause of ESRD and its impact on mortality during the study period, adjusted for potential confounders. Of the 7,732 patients identified with HIVAN, only 60 were younger than 21 years and were classified as children; 50% were males and the majority (88.3%) was black. The cumulative percentage survival of children with HIVAN at 12, 24, and 36 months was better than adults with HIVAN (76%, 62%, and 54% vs. 60%, 43%, and 34%). Survival of children with HIVAN who started dialysis after 1996 was significantly better than those who started dialysis in or before 1996 (log rank P value <0.043). However, the major factor associated with better survival on Cox proportional hazard analysis was female gender (male vs. female AHR 2.85, 95% confidence interval 1.04-6.73). We conclude that only a small number of children with HIVAN and ESRD have received dialysis in the United States. The prognosis of these children is better than that of adults with HIVAN and among children with HIVAN females have better survival than males.