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BACKGROUND: The Susac syndrome is often misdiagnosed as Multiple Sclerosis. In the absence of an early diagnosis of the disease, both the doctors and patients concerned are left in an uncertainly regarding the interpretation, diagnosis and prognosis of the discovered illness. PATIENT: The following case report focuses on this syndrome. CONCLUSION: The disease mainly affects young women and appears in the triple form of retinal arterial occlusion, hearing impairment and Encephalopathy.