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Budd-Chiari syndrome diagnosed with pregnancy in a patient with inherited thrombophilia. An 18-year-old primigravida was referred to our high risk pregnancy (HRP) department at 34 weeks of gestation for birth panning as she has Budd-Chiari syndrome (BCS). Her history was significant for familial thrombophilia. She had portal hypertension manifestations. Her work-up revealed factor V Leiden gene mutation, hepatic and portal vein thrombosis. A multidisciplinary team of physicians from the gastroenterology and hepatology, haematology and HRP departments puts a management plan; it culminated into safe delivery of the patient at 36 weeks of gestation. The patient was referred to a specialised BCS centre where she had successful liver transplantation done.