Objective To investigate the outcome of the surgical treatment for congenital blepharophimosis syndrome. Methods This was a retrospective case study to analyze a treatment approach for congenital blepharophimosis syndrome and its clinical efficacy. A bilateral correction of the epicanthus and blepharoptosis was performed simultaneously. Suitable methods were adopted to correct the epicanthus: Y-V, Stallars "Z" , Speath and classic Mastands. Twenty-three patients (46 eyes) were involved in the study, ranging in age from 2 to 11 years. All patients suffered from eye or systemic disease. There were cases of strabismus, refractive error, amblyopia, inherited and systemic disease. The study analyzed effectiveness by comparing the preoperative and postoperative status of palpebral fissure length, palpebral fissure width, inner canthic diameter and eyebrow-eyelid diameter using a paired t-test. Patients were followed up for 1 to 24 months after the operation. Results There was an obvious improvement in palpebral fissure length and palpebral fissure width in all patients, and an obvious decrease in the inner canthic diameter and eyebrow-eyelid diameter. Comparisons one month after the operation showed that palpebral fissure length changed from (18.5±1.1)mm to (23.4±1.2)mm, palpebral fissure width changed from (2.1±1.2)mm to (6.7±0.9)mm, inner canthic diameter changed from (35.2±1.4)mm to (31.1±1.1)mm, and eyebrow-eyelid diameter changed from (13.6±1.3)mm to (9.8±1.2)mm. The differences were statistically significant (P<0.05). There was only one case with complication of exposure keratitis after the operation. Conclusion For congenital blepharophimosis syndrome with refractive errors and amblyopia, it is better to perform a bilateral correction of the epicanthus and blepharoptosis simultaneously, which is a more ideal approach than other treatments.