Entropion is a malposition of the eyelid causing an abnormal inversion of the eyelid margin. This results in symptomatic contact of the eyelashes with the surface of the globe, resulting in corneal irritation. Symptoms primarily arise from irritation of the ocular surface. Corneal abrasions and scarring can occur. Although common in many canine breeds, it is rare in the pediatric congenital form and rarely reported in the literature. Success in treatment depends on diagnosing the appropriate cause and selecting a surgical procedure that adequately addresses the underlying abnormality. The authors describe a pediatric patient with congenital entropion and review the nature of the problem and its treatment options.