Purpose To study the safety and efficacy of posterior scleral reinforcement (PSR) combined with phakic intraocular lens (PIOLs) implantation for highly myopic amblyopia in children. Methods This study included eight highly myopic children (11 eyes) who failed in conventional therapy for amblyopia using various combination of spectacles, contact lenses, and intensive patching before enrollment into this study. They were treated sequentially with PSR and PIOL implantation, and were followed up for 3 years after surgery. Uncorrected visual acuity (UCVA) and best corrected visual acuity (BCVA) in LogMAR, spherical equivalent power (SE), and complications were evaluated. Results Before surgery, the mean UCVA was 1.59 ± 0.33, BCVA, 0.74 ± 0.37, SE, À 17.57 ± 5.56D, the axial length (AL), 30.09±2.18 mm. After PSR, BCVA improved one line in three patients, the rest were unchanged, and AL was unchanged among all cases. Six eyes of three patients were implanted with an iris-claw PIOL and five eyes of five patients were implanted with a posterior PIOL. After completion of treatment, the mean UCVA was 0.44 ± 0.21, BCVA 0.38±0.24, SE À 0.54±0.74 D, and AL 30.35±2.29 mm. No patient experienced complications. Conclusion Combined PSR and PIOL implantation treatment for highly myopic amblyopia in children is safe and effective.