This is a case report of Bernard Soulier syndrome with pregnancy. This syndrome was first described in 1948 and is a rare autosomal recessive disorder characterized by platelet dysfunction. There is mild thrombocytopenia and a failure of platelets adhesion to the vessel wall. Our case report is the 12th case reported in the literature, and the first to be complicated by pre-eclampsia. Bernard-Soulier syndrome results in considerable morbidity due to haemorrhage and isoimmunization. Preeclampsia complicating the pregnancy, as in our patient, may be a diagnostic dilemma in uninvestigated cases, where low platelets may simulate HELLP syndrome, moreover the two conditions together can complicate the situation further. Since there are no clear cut guidelines available for its management we suggest some basic principles for the prevention of complications and management of this syndrome.

Keywords: Bernard soulier syndrome, Pregnancy induced hypertention, Pregnancy