Congenital eversion or ectropion of the upper eyelids is rare. Only 50 cases have been reported since the condition was first described in 1896. We encountered an additional case of congenital ectropion of the eyelids, which is also unusual because of its late presentation for treatment. This case demonstrates the unfavorable outcome of failure to treat this condition at birth and illustrates the association with Down syndrome. Our case is compared with the others reported in the literature.