Congenital absence of the portal vein in oculoauriculovertebral dysplasia (Goldenhar syndrome).

Absence of the portal vein with systemic visceral venous return was demonstrated in an 8-year old girl with oculoauriculovertebral dysplasia (Goldenhar syndrome) during preoperative evaluation of a liver mass. Congenital absence of the portal vein is a rare malformation of potential clinical significance.