This study aimed to report the efficacy and outcome safety of capsular tension segment (CTS) combined with capsular tension ring (CTR) in ectopia lentis in children. This retrospective case series included patients with nontraumatic ectopia lentis under 18 years old who underwent intraocular lens (IOL) implantation with CTS and CTR combined between 2022 and 2023. The primary outcomes were best-corrected visual acuity (BCVA), IOL centration, and postoperative complications. Four patients, aged 6, 9, 10, and 13, had bilateral subluxated lenses and underwent the surgery. Ectopia lentis with a suspicion of Marfan syndrome was established as the diagnosis. Improvement of the BCVA and IOL centration was seen in all the patients. Posterior capsular opacification occurred in one patient, who was then treated with Nd: YAG capsulotomy. IOL implantation with a combination of CTS and CTR showed a promising visual outcome in children with ectopia lentis.