Background: Susac syndrome, a rare disease is characterized by a clinical triad of subacute encephalopathy, visual loss secondary to retinal branch occlusions and sensorineuronal hearing loss. Case Report: We hereby report a 25 years old pregnant (7 months amenorrhea) female who presented with loss of vision of the left eye, tinnitus of the right ear and mild headache. Fundus evaluation showed branched retinal arterial occlusions in the left eye. A mild-moderate intensity right sided sensorineural hearing loss was observed on audiometry. Magnetic resonance images showed a hyper-intense lesion in the body of corpus callosum and peri-ventricular region. Patient was well managed with high dose steroids. Conclusion: A high index of suspicion of Susac syndrome is important as timely administration of immunosuppressive treatment may prevent permanent neuropsychological or visual sequelae.