Susac syndrome (SS) consists of a triad of vision loss, hearing loss, and encephalopathy due to autoimmune-mediated vascular endothelial injury. Herein we describe a 25-year-old previously healthy woman who presented at 20 weeks' gestation with symptoms of confusion, difficulty walking, and vision and hearing loss. She had branch-retinal artery occlusions on funduscopic examination, and sensorineural hearing loss. Additionally, non-contrast enhanced brain magnetic resonance imaging showed multiple white matter and callosal lesions consistent with ischemia. She was treated initially with aspirin, corticosteroids, and intravenous immunoglobulin with early improvement, although recurrent disease was treated with cyclophosphamide and rituximab after induction of premature delivery (at 35 weeks' gestation) to spare the fetus possible toxicity. We additionally discuss a general overview of SS, what is known about pregnancy and this disease, and issues regarding diagnostic and treatment approaches for SS during pregnancy.