Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare autosomal-dominant disease. It usually requires early surgical intervention to prevent amblyopia in children and to address other functional concerns in aged patients. We report the case of an 87-year-old woman with BPES, without previous surgical intervention, who underwent levator muscle advancement in both eyes. A satisfactory outcome and an improved quality of life were attained.