Susac's syndrome is a rare disease characterized by encephalopathy, retinal artery occlusion, and a sensorineural hearing loss. Diagnosis may be difficult since most specialists are not familiar with this angiopathy. However, the typical symptom complex can mimic different pathologies, therefore requiring the attention of radiologists, ENT specialists, and ophthalmologists. We present a rare case of Susac's syndrome unveiled by audiometry results, MR imaging of the brain, and the ophthalmological findings.