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Background and Aims Pulmonary hypertension (PH) is associated with increased maternal mortality during pregnancy, particularly when secondary to congenital heart disease. The coexistence of Factor V Leiden mutation further raises the risk of thromboembolic complications, making anesthetic management highly challenging. This case report aims to highlight the multidisciplinary perioperative approach used in managing a pregnant patient with PH and thrombophilia undergoing caesarean section.Methods A 32-year-old primigravida at 37+1 weeks gestation with a history of surgically repaired Tetralogy of Fallot was scheduled for elective caesarean delivery. She was diagnosed with PH, Factor V Leiden mutation, immune thrombocytopenic purpura, and portal hypertension. Preoperative assessments included echocardiography showing severe pulmonary-tricuspid regurgitation and an estimated pulmonary artery pressure of 33 mmHg. After cardiology and hematology consultations, thromboprophylaxis and infective endocarditis prevention were planned. Epidural anesthesia was chosen to maintain hemodynamic stability. Invasive arterial monitoring was implemented, and 12 mL of 0.5% bupivacaine with 100 mcg fentanyl was administered epidurally.Results Intraoperative hemodynamics remained stable without complications. The patient was monitored in the intensive care unit for 24 hours. Transient left thigh numbness was observed and resolved after epidural catheter removal, which was performed when platelet count exceeded 70,000/mm 3. No thromboembolic or bleeding events occurred. Both mother and newborn were discharged in good condition.Conclusions This case underscores the importance of individualized anesthetic planning and multidisciplinary coordination in pregnant patients with PH and thrombophilia. Epidural anesthesia can be safely utilized with favorable maternal and fetal outcomes when carefully executed.