IntroductionThe first case reports of Susac Syndrome emerged in 1979 due to two young female patients presenting with the clinical triad of multifocal encephalopathy, occlusion of the central retinal artery branch, and sensorineural hearing loss, as well as histopathological findings of microinfarctions in the gray and white matter of the brain. The clinical features of the syndrome have been documented in approximately 500 cases published worldwide, making it extremely rare. Headache is a prominent complaint by patients with Susac syndrome. Its pathophysiology remains unknown, with immune and genetic factors being most related to the retino-cochleo-cerebral clinical manifestations. Since its first description, the scientific community has been paying greater attention to the syndrome, especially with advances in medical technology and the availability of complementary exams.Case reportIn this study, We present a case report of a woman experiencing progressive pulsatile temporal headaches for two years, associated with decreased visual acuity for three months and simultaneous hypoacusis on the left, who was incorrectly diagnosed and treated for multiple sclerosis.CommentThe literature review discusses Susac Syndrome and its microvascular complications, highlighting the multidisciplinary approach necessary for accurate diagnosis and comprehensive long-term management.