Background and objectives Poor linear growth is common in children with CKD and has been associated with higher mortality. However, recent data in adult dialysis patients have suggested a higher risk of death in persons of tall stature. In this study, we aimed to examine the risk of all-cause and cause-specific mortality in children at both extremes of height at the time of first RRT.Design, setting, participants, & measurements Using the US Renal Data System, we performed a retrospective analysis of 13,218 children aged 2-19 years, who received their first RRT (dialysis or transplant) during 1995-2011. We used adjusted Cox models to examine the association between short (,3rd percentile) and tall (.3rd percentile) stature and risk of death, compared with less extreme heights.Results Over a median follow-up of 7.1 years, there were 1721 deaths. Risk of death was higher in children with short (hazard ratio, 1.49; 95% confidence interval, 1.33 to 1.66) and tall stature (hazard ratio, 1.32; 95% confidence interval, 1.03 to 1.69) in adjusted analysis. In secondary analyses, there was a statistically significant interaction between height and body mass index categories (P=0.04), such that the association of tall stature with higher mortality was limited to children with elevated body mass index (defined as $95th percentile for age and sex). Children with short stature had a higher risk of cardiac-and infection-related death, whereas children with tall stature had a higher risk of cancer-related death.Conclusions Children with short and tall stature are at higher mortality risk, although this association was modified by body mass index at time of first RRT. Studies to further explore the reasons behind the higher risk of mortality in children with extremes of height at the time of first RRT are warranted.