We report the case of a surgically treated congenital Budd-Chiari syndrome related to agenesia of the retrohepatic inferior vena cava. The first symptoms of the disease were noticed in childhood. The increasing symptomatology led to propose, at the age of 30 years, first a percutaneous transluminal angioplasty which failed, because of the impossibility to recanalize the obstructed segment. Then a surgical procedure consisting of the implantation of ePTFE prosthesis between the right atria and the retrohepatic inferior vena cava was performed. The hepatic biopsy showed a centrolobular fibrosis and an old subglissonian infarction. The patient was improved, allowing him to recover a normal life. However, three years later, an angiographic evaluation performed because of a recurrence of a slight abdominal pain, showed a thrombosis of the bypass. An attempt at thrombolysis failed. Since the patient did not present major clinical and biological consequences we only proposed a surveillance and no endovascular procedure because of the fear of a pulmonary emboli. The purpose of this case report is to review the literature and discuss the etiopathology of congenital Budd-Chiari syndrome with regard to the different therapeutic options.