Background and Clinical Significance: Budd–Chiari syndrome (BCS) is a rare disorder characterized by hepatic venous outflow obstruction, often associated with underlying hypercoagulable states. Pregnancy represents a physiologic prothrombotic condition that may precipitate disease onset. Case Presentation: We report a case of de novo BCS diagnosed in the second trimester in a previously healthy 36-year-old multiparous patient. Evaluation revealed cirrhotic liver morphology, portal hypertension, and bleeding esophageal varices requiring emergent treatment. The patient’s course included a transjugular intrahepatic portosystemic shunt (TIPS) procedure with complications, anticoagulation, multidisciplinary care, and a work-up revealing a JAK2 mutation consistent with an underlying myeloproliferative disorder. Despite apparent maternal stabilization and reassuring fetal growth, the pregnancy resulted in intrauterine fetal demise at 34 weeks’ due to placental abruption, followed by postpartum hemorrhage. Conclusions: This case highlights the diagnostic and therapeutic challenges of BCS in pregnancy, and demonstrates that favorable maternal stabilization does not preclude severe obstetric complications.