AIM:To explore the one-stage repair effect of congenital blepharophimosis syndrome by combining Y-V medial canthoplasty with the frontalis suspension using dacron mesh sling . METHODS:From 2004 to 2009, an one-stage procedure combining blepharoptosis correction with medial canthoplasty was adopted in 14 patients with congenital blepharophimosis syndrome (5 male, 9 female; aged from 4 to 14). Bilateral severe blepharoptosis and epicanthus inversus were found in all the patients, 12 (86%)patients had flat dorsum nasi. The vertical length of the palpebral fissure was 1 to 5mm, horizontal length 18 to 25mm, and the intercanthal distance was 38 to 43mm. RESULTS:In the follow-up periods ranged from 1 to 24 mo, in all 14 cases, the vertical length of the palpebral fissure was 5 to 9 mm, horizontal length was 25 to 30mm, and the intercanthal distance was 30 to 35mm. The appearance of their bilateral eyelids was satisfying. CONCLUSION:The modified one-stage procedure combining Y-V medial canthoplasty with the frontalis suspension using dacron mesh sling can achieve favorable outcomes for patients with congenital blepharophimosis syndrome.