Objective To explore the clinical effect of combining medial and lateral canthoplasty with blepharoptosis correction at onestage for congenital blepharophimosis syndrome. Methods From January 2002 to May 2006, 26 patients(52 sides) with congenital blepharophimosis syndrome were treated. There were 16 males and 10 females, aging from 3 to 35 years (mean 8.5 years). They were all bilateral blepharoptosis significantly. The palpebral muscle force was 03 mm; the transverse dimension and vertical dimension of the palpebral fissue were 13-22 mm and 2-4 mm; the intercanthal distance was 33-44 mm; the levator function was 1-3 mm. Results Twenty-six patients underwent medial canthoplasty and blepharoptosis correction, of them, 12 patients were also given lateral canthoplasty at one-stage. The postoperative transverse dimension and vertical dimension of the palpebral fissue were 6-8 mm and 24-32 mm, respectively. The intercanthal distance was 29-34 mm. The levator function was 46 mm. The supratarsal fold in the upper lid was natural. With a follow up of 3 months to 4 years，all patients were satisfied with their results. Conclusion One-stage surgical treatment of combining medial and lateral canthoplasty with blepharoptosis correction can achieve good result for blepharophimosis syndrome with a shortened treatment time.