A case of primary congenital upper eyelid entropion ( PCUEE ) is reviewed. The author proposes a congenital shortness of the levator muscle with anomalous insertion onto the tarsal plate as the etiology, and a new simplified surgical correction is described. The classification of PCUEE as a syndrome with multiple congenital anomalies is supported, and the need for a complete systemic evaluation is stressed. Congenital primary upper eyelid entropion is an exceedingly rare condition. In 1969, Hiles and Wilder reviewed the 13 known cases in the ophthalmic literature, and presented a new case. They suggested the possibility that congenital upper eyelid entropion was part of a syndrome involving multiple systemic anomalies. Surgical correction of the ectropion is usually necessitated by corneal erosion secondary to trichiasis, and a multifarious assortment of surgical procedures has been described. The following is a case report of primary congenital upper eyelid entropion which corroborates the categorization as a syndrome, suggests a pathophysiologic abnormality of the eyelid as the etiology, and describes a new, simplified technique of surgical repair.