Susac syndrome is an extremely rare disease that may present with ophthalmological, audiological, and neurological manifestations, typically affecting young women. Among the approximately 400 cases described in the literature, 5% involve women during pregnancy or the postpartum period. The classic triad of symptoms includes encephalopathy, visual disturbances caused by occlusion of the retinal artery branches, and sensorineural hearing loss, typically bilateral. The diagnostic process involves evaluation of the clinical picture, magnetic resonance imaging, lumbar puncture, and audiological tests. Treatment should be tailored to pregnant individuals; typically, it is based on intravenous steroids, intravenous immunoglobulins, oral steroids, or plasma exchange. It is vital to take into consideration the teratogenicity of drugs and their effect on the foetus. Due to the varied clinical presentations of Susac syndrome, patients are often misdiagnosed or underdiagnosed, and late implementation of treatment may lead to irreversible hearing loss or blindness. The literature shows that most pregnancies affected by Susac syndrome lead to safe delivery of healthy newborns. However, due to the lack of standardised recommendation regarding this condition, further research is needed to establish standards of treatment. The primary limitation for expanding research is the rarity of the disease. The main aim of the study is to summarise recent knowledge about Susac syndrome in pregnant women.