Budd-Chiari syndrome is a rare and serious thrombotic event with significant morbidity and mortality. Recommendations regarding future conception and management during pregnancy have not been defined. We present a patient with history of idiopathic Budd-Chiari Syndrome and subsequent orthotopic liver transplantation who was successfully managed during pregnancy. A 24-year-old white female, gravida 1 para 0, status postorthotopic liver transplantation 5 years previously for Budd-Chiari syndrome with post-transplant insulin-dependent diabetes mellitus presented to our clinic at 7 weeks of gestation for initial prenatal evaluation. Maintenance immunosuppressive therapy and prophylactic heparin anticoagulation was administered throughout the pregnancy, which was uneventful until 35 weeks gestation, at which time pre-eclampsia and premature preterm rupture of membranes prompted labor induction. The patient developed no evidence of acute or chronic hepatic rejection and no evidence of recurrent Budd-Chiari syndrome during the pregnancy or post-partum convalescence. Prudent use of prophylactic anticoagulation, close immunosuppressive monitoring, and periodic fetal and maternal surveillance are warranted in patients with previous orthotopic liver transplantation for idiopathic Budd-Chiari syndrome and may reduce risk of recurrence during pregnancy.