HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome is a variant of preeclampsia syndrome and is considered a life-threatening complication of pregnancy with a significant chance of preterm delivery and a high fatality rate. It is typically diagnosed in the third trimester of pregnancy and can have a variable presentation when compared to typical preeclampsia. Laboratory and imaging studies can help with narrow the differential diagnosis, assess disease progression, and identify complications. Prevention in future pregnancies is geared toward modifiable risk factors, as most other modalities have not been shown to be useful. Research is aimed at identifying methods to prevent disease development, reduce disease progression, and identify those most likely to develop HELLP syndrome.