We report a case of posterior reversible encephalopathy syndrome in association with hemolysis-elevated liver enzymes-low platelet counts (HELLP) syndrome. A 29-year-old primigravida presented at 35 weeks 4 days pregnancy with headache and blurred vision and the laboratory data revealed hemolysis, elevated liver enzymes, and low platelets. Cranial magnetic resonance imaging (MRI) showed hyperintense signal changes in the right occipital, left cerebellar regions and the right thalamus. Follow up brain MRI 10 days after the caesarean section showed resolution of the previous abnormalities. Posterior reversible encephalopathy syndrome (PRES) is a neuroradiologic condition associated with headache, seizures, altered sensorium, and visual disturbances. Although their association is rare, the diagnosis of PRES should be considered in all HELLP patients presenting with headache and neuro-ophthalmic signs. It is for the reason that the patients can recover well with complete reversal of clinical symptoms within several days, when adequate treatment is immediately initiated.