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Chicken ovalbumin upstream promoter-transcription factors (COUP-TFs) are the orphan receptors of the steroid/thyroid hormone receptor superfamily. These factors include COUP-TFⅠ and COUP-TF Ⅱ, which play critical roles in embryonic development, organogenesis, neurogenesis and cellular differentiation. Many studies showed that COUP-TFs were also involved in the development of embryonic eye. More attention has been paid to the issue that mutations of COUP-TFs genes lead to phenotypes of coloboma, small eyes, optic atrophy, eye retardation, strabismus, and amblyopia. However, the mechanisms of COUP-TFs regulating the development of embryonic eye are not clear. The origin, molecular struture, classification, biological functions, mechanisms of regulating the target genes transcription and development of embryonic eye of COUP-TFs were reviewed.