HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome is a variant of severe preeclampsia which is associated with substantial maternal and perinatal morbidity and mortality. As with preeclampsia, the etiology and pathogenesis of HELLP syndrome is not completely understood. An increase in vascular thrombosis and activation of the coagulation system may be important in the clinical presentation of this disorder. Laboratory criteria for the diagnosis of HELLP syndrome have been classically described but lack uniformity among different institutions. Aggressive management of HELLP syndrome with expeditious delivery appears to yield the lowest perinatal mortality rates. Conservative or expectant management has been associated with higher stillbirth rates with antenatal corticosteroids not causing resolution of the laboratory abnormalities. Resolution of laboratory abnormalities in HELLP syndrome runs a protracted course over several days after delivery. Despite nearly two decades since HELLP syndrome has been defined as a clinical entity, treatment for the disorder still remains delivery of the patient.