To retrospectively analyze the clinical efficacy of an innovative mitral valvuloplasty strategy in the treatment of mitral regurgitation in children. From January 2018 to December 2022, 140 patients undergoing surgical treatment for mitral regurgitation in our hospital were enrolled. Ninety patients underwent three-step standardized pediatric mitral valvuloplasty (group A) and 50 patients underwent simple annuloplasty (group B). The incidence of primary and secondary study endpoint was compared between the two groups, and the independent risk factors for the primary study endpoint were determined. Our primary study endpoint was a composite endpoint of postoperative functional mitral failure, postoperative heart failure, transplantation, and/or mortality. Secondary end points were defined as perioperative complications. During the follow-up period, there was no all-cause death. Primary endpoint events occurred in 22 patients, including 12 patients in group A and 10 patients in group B. There was no significant difference in the incidence of primary and secondary endpoint events between the two groups. Multivariate Cox proportional hazards regression analysis showed that younger age and residual mitral regurgitation at discharge were independent risk factors for the primary endpoint events, while type of MV repair was not an independent risk factor. Subgroup analysis based on age showed that primary endpoint events occurred in 4 patients in group A and 7 patients in group B in patients < 1 year old. The incidence of primary endpoint events in group A was lower than that in group B (6.06% vs. 20.59%, P = 0.041). In patients ≥ 1 year old, the primary endpoint event occurred in 8 cases in group A and 3 cases in group B. There was no significant difference in the incidence of primary endpoint events between groups A and B (33.33% vs. 18.75%, P = 0.312). The degree of mitral regurgitation at discharge was significantly improved compared with that before operation in both groups (P < 0.001), and the degree of mitral regurgitation at the last follow-up was not significantly worse than that at discharge (P = 0.090). The mid-term results of mitral valvuloplasty for mitral regurgitation in children are encouraging. The perioperative recovery and postoperative outcomes of three-step standardized mitral valvuloplasty in children are not inferior to those of annuloplasty alone. Three-step standardized pediatric mitral valvuloplasty has better postoperative outcomes than simple mitral annuloplasty, especially for patients younger than 1 year old. Children with residual mitral regurgitation at discharge should be followed up regularly to be alert to the occurrence of poor prognosis.