The coincidence of hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome and cortical blindness is an uncommon but very dramatic event. We describe a case of HELLP syndrome complicating with acute cortical blindness before delivery. A 27 year-old woman, gravida 1, para 0, with normal medical history, was referred to our emergency department at the 33th week of gestation due to headache, vomiting, and blurred vision. The ophthalmologic examination showed intact pupillary light reflexes and normal ophthalmoscopic findings, but no light perception in either eye. Brain computed tomography showed normal findings. HELLP syndrome and preeclampsia was diagnosed based on the findings of hypertension and proteinuria as well as laboratory data. Prompt delivery was performed in order to achieve good maternal and neonatal outcomes.