Children and adolescents with elevated body mass index (BMI) who have normal waist circumference (NWC) have a cardiometabolic risk profile similar to normal children. However, there is a lack of adequate information regarding their left ventricular mass (LVM). The present study was undertaken to evaluate LVM in children with elevated BMI with NWC. LVM was assessed by echocardiography in 247 children (age 2 to 19 years) without evidence of heart disease. Data on those who had elevated BMI with NWC (group 1, n = 80) were compared with matched normal controls with normal BMI who had NWC (group 2, n = 80) and children with elevated BMI with increased waist circumference (IWC; group 3, n = 87). Correlations, t tests, and linear regressions were used for statistical testing. LVM in children with elevated BMI with NWC was not significantly different from normal controls (97.6 ± 44.4 vs 100.7 ± 47.9 g, p = 0.6713, respectively); however, it was significantly less than that in subjects with elevated BMI who also had IWC (97.6 ± 44.4 vs 114.5 ± 47.8 g, p = 0.0193, respectively). Similar to normal controls, those subjects with elevated BMI with NWC had a stronger correlation between LVM and lean body mass (R(2) = 0.86 and 0.86, respectively) than subjects with elevated BMI with IWC (R(2) = 0.75). In conclusion, children with elevated BMI with NWC appear to have a similar LVM profile as children with normal BMI with NWC. The present study emphasizes the importance of measuring waist circumference in children with elevated BMI.