Background <p>Pediatric fragmental distal femoral fractures present significant clinical challenges for pediatric orthopedic surgeons owing to their propensity for severe posttreatment complications, notably growth disturbances, and angular deformities. A comprehensive review of current treatment modalities and associated secondary deformities is essential to optimize management strategies and improve long-term functional outcomes in this pediatric population.</p> Case presentation <p>The patient was a 6-year-old Chinese boy with a known case of fragmented fracture of the distal femur involving the growth plate developed in a car accident, which had been diagnosed on the basis of X-rays. After the patient was injured, open reduction and combined external fixation were performed. A secondary deformity occurred after removal of the external fixator as the bone later became bowl-like. Femoral osteotomy was performed using a locking plate to reconstruct the lower limb mechanical line. The release of soft tissues and knee joint stiffness was also completed simultaneously. The patient subsequently recovered well.</p> Conclusion <p>We conducted a systematic review of the management strategies for pediatric fragmental distal femoral fractures complicated by secondary deformities, incorporating a literature search of PubMed-indexed studies. Our analysis underscores the necessity of prioritizing growth plate preservation during initial treatment. Critical postoperative outcomes include restoring knee range of motion and achieving symmetrical femoral length recovery, which are paramount for minimizing long-term functional deficits.</p>