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In congenital eyelid imbrication syndrome, a rare condition, the upper eyelids override the lower lids. It has been described in the literature either as an isolated entity or associated with floppy eyelid syndrome. We report the case of a 2-month-old with congenital eyelid imbrication syndrome associated with complex microphthalmos in the same eye and persistent fetal vasculature in the other eye, with no other systemic abnormality. The eyelid condition improved spontaneously over a period of 2 months. The persistent fetal vasculature