Background: The relationship of Haemolysis, Elevated Liver Enzymes and Low Platelets (HELLP) syndrome with maternal and perinatal health and its presentation in Pakistani population is not known.Purpose: To determine the mode of presentation along with maternal and perinatal outcome of patients with HELLP syndrome.Methods: Case records of patients with severe hypertension in pregnancy who delivered between January 1, 1989 and December 31, 1994 at The Aga Khan University Hospital, Karachi. Out of 120 cases of severe pre-eclampsia/eclampsia, there were 36 cases of HELLP syndrome (Group-A). These were then compared with cases without HELLP syndrome (Group B) for their mode of presentation along with maternal and perinatal morbidity and mortality.Results: The overall incidence of HELLP syndrome was 0.4%. In the antepartum factors; unbooked status (66% vs 30%; p \u3c 0.05), diastolic B.P. \u3e 120 mmHg (61% vs 16%; p \u3c 0.05) DIC (13% vs 2%; p = 0.03), seizures (40% vs 16%, p = 0.01) and ARF (11% vs 1%, p = 0.07) were significantly raised. In the intrapartum factors there were no significant differences between the two groups in mode of delivery and complications of delivery. Neonatal outcomes did not differ significantly in the two groups.Conclusions: Women with severe hypertension in pregnancy manifesting with HELLP syndrome show a significantly greater frequency of developing DIC, seizures and acute renal failure. Therefore, their care necessitates intensive monitoring to preclude development of these complications