De Morsier`s Syndrome Expressed as Congenital Exotropia

De Morsier syndrome, or septo-optic dysplasia is a developmental anomaly characterized by unilateral or bilaterral optic nerve hypoplasia, agenesis of septum pellucidum, visual disturbance, and a variable spectrum of disturbed hypothalamic-pituitary function. We experienced a 7-month-old female with de Morsier syndrome whose presenting manifestation was congenital exotropia of right eye, and subsequent evaluation revealed optic nerve hypoplasia of right eye, absence of septum pellucidum, and hypothyroidism.