The ventricular remodeling that occurs in adults with left ventricular (LV) dysfunction is characterized by a change in LV shape from an ellipse to more of a sphere and is associated with increased functional mitral regurgitation (MR), decreased exercise tolerance, and poor outcome. There are limited data on the occurrence and importance of LV remodeling in children with LV dysfunction. The purposes of this study were to evaluate in children with LV dysfunction (1) LV shape, (2) changes in LV shape as LV function improves, and (3) the relation between LV shape and functional MR. Children with LV dysfunction were identified and compared with controls. Patient demographics, treatment, and outcomes were noted. Echocardiograms were reviewed for LV function and shape (sphericity index). The echos of a subset of children whose LV ejection fractions (LVEFs) increased by >10% over a follow-up of >6 months were analyzed for changes in sphericity, mitral annulus size, and the degree of MR. Twenty-five children with LV dysfunction were compared with 37 age-matched controls. LV remodeling to a more spherical shape was inversely related to LVEF but was not associated with clinical outcome. In a subset of patients (n = 17) whose LVEFs improved, the reversal of remodeling (decrease in sphericity index) was associated with a decrease in MR and mitral annulus size. In conclusion, LV remodeling occurs in children with LV dysfunction. The reversal of this remodeling with a decrease in the degree of MR and the size of the mitral annulus occurs as systolic function improves.