Congenital eversion of the eyelids is a rare condition which has never had a proven anatomic etiology. A one-day-old male presented with severe congenital eversion of both upper eyelids. Medical therapy was unsuccessful. At surgery, adipose tissue was found beneath the tendon of the levator palpebrae and extended anterior to the tarsus because the orbital septum was not attached inferiorly. The defect was repaired. In this case, non-attachment of the orbital septum may have induced eversion of the upper eyelids.