A case of Susac syndrome in a child is reported with a review of the literature. A 14-year-old girl presented with headache, left hemiparesis, sphincter deficit, and cognitive deficits. The assessment consisted of neurological and ocular examination, imagery by cerebral magnetic resonance, lumbar puncture, and a biological and immunological assessment. Magnetic resonance imaging revealed numerous high signal intensities on T2-weighted images in the white and grey matter. The ophthalmologic examination found bilateral branch retinal artery occlusions. Twelve months later, she developed hearing loss. Infection assessment was negative and immunologic tests were normal. Susac syndrome is a rare disorder with a clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. It is caused by a microangiopathy affecting the precapillary arterioles of the brain, retina, and inner ear. Susac syndrome usually affects young women in young adulthood and is extremely rare during childhood. The pathogenesis of this syndrome is unknown. The clinical course of Susac syndrome is characterized by recurrent attacks. Resolution usually occurs spontaneously. However, sensory and neurologic sequelae may be present. Treatment is not well codified and may include steroids, immunosuppressant drugs, and immunoglobulin. Susac syndrome should be considered in children when evaluating patients with branch retinal artery occlusion and encephalopathy.