HELLP syndrome – a severe pregnancy complication associated with hypertension, is characterized by hemolysis, elevated liver enzymes, and low platelets. The condition increases the risk for perinatal complications for the mother and offspring. The prevalence of HELLP syndrome is 0.2-0.8%, which often coexists with preeclampsia. Both preeclampsia and HELLP syndrome have a genetic predisposition while a history of HELLP pregnancy increases the preponderance of developing this entity in future pregnancies. Although a single genetic cause is yet to be identified, multiple gene variants imposing moderate risks along with maternal and environmental factors have been implicated in the etiology of HELLP.  The current review underscored the pathophysiological mechanism of HELLP syndrome. A better knowledge of the disease course is essential for its early detection, accurate diagnosis, and appropriate management of this condition. Source: Medicina (Kaunas). 2022 Feb 21;58(2):326. doi: 10.3390/medicina58020326.