HELLP syndrome is a disorder associated with serious maternal morbidity and mortality. Distinguishing HELLP from other pregnancy-related disorders is often challenging and may result in delay of treatment. Differential diagnoses include acute fatty liver of pregnancy, thrombotic thrombocytopenic purpura, antiphospholipid syndrome, and hemolytic uremic syndrome, and are reviewed in this chapter. While there is not any current treatment for HELLP, the mainstay of treatment involves maternal stabilization and timely delivery. Various treatment strategies have been attempted to help decrease the morbidity and mortality of HELLP, including the maternal use of corticosteroids. The authors review the studies and controversies surrounding the maternal use of corticosteroids, plasma exchange, and low molecular weight heparin for the treatment of HELLP, as well as the role of the complement system in HELLP. Further large, well-designed, randomized controlled trials are needed to address the role corticosteroids may play in the treatment of women with HELLP and to help improve maternal and fetal outcomes.