BCS Budd Chiari syndrome HTT Hereditary hemorrhagic telangiectasia TIPS transjugular intrahepatic portosystemic shunt Among patients with Budd Chiari syndrome (BCS), portal vein thrombosis and porto-sinusoidal vascular disease, women of childbearing age account for 50%, 20% and 15%, respectively [1-3]. The affected women in this age have gained a long life expectancy since anticoagulation therapy, followed in specific cases by transjugular intrahepatic portosystemic shunt (TIPS), angioplasty or liver transplantation have been applied. Indeed, with an appropriate management, five-year survival rate is currently above 90% for women with BCS or portal vein thrombosis, and 85% for porto-sinusoidal vascular disease [1,2,4]. It is therefore not surprising that affected women with a well-controlled disease express a desire for pregnancy.