Sitosterolaemia is a rare metabolic disorder characterised by excessive accumulation of plant sterol in the blood and presents with accelerated atherosclerosis, macrothrombocytopaenia, splenomegaly, haemolytic anaemia and liver cirrhosis. It is under-recognised but prevalence may be as high as 1 in 50000. Management involves dietary avoidance of plant sterols, and Ezetimibe. To our knowledge, no prior case reports exist describing pregnancy outcomes in women with sitosterolaemia. We describe a 22-year-old primigravida with progressive thrombocytopenia and liver function derangement in pregnancy. At 6 weeks, our patient had a platelet count of 52 x10^9/L, mild anaemia and splenomegaly. Ezetimibe was initially stopped for the first trimester. By third trimester, platelet count was 33 x10^9/L without bleeding, and she developed liver function derangement. She was thought to have preterm premature rupture of membrane at 32 weeks (later dismissed in the pursuing weeks). Due to concerns of early delivery, multiple treatments were trialled including intravenous immunoglobin, platelet transfusions and recommencement of Ezetimibe. However the patient’s thrombocytopenia remained refractory. Thromboelastography showed normal coagulation. The multidisciplinary team decision was to await spontaneous labour to avoid unnecessary obstetric interventions. Spontaneous labour started at 37 weeks with delivery by emergency caesarean section with general anaesthesia. Minimal operative bleeding. Following the birth, there was improvement of her platelet count and liver function. This case highlights two points: the value of a multidisciplinary team in the management of rare metabolic conditions in pregnancy, and the possibility of delivery with a lower platelet count in patients with stable non-consumptive refractory thrombocytopenia.