Congenital absence of the portal vein with systemic visceral venous return in a 8-year-old girl with oculoauriculovertebral dysplasia (Goldenhar Syndrome) had been previously reported following its discovery during preoperative evaluation of a liver mass which was diagnosed as focal nodular hyperplasia after open biopsy. Subsequently, an enlarging diffuse hepatic neoplasm developed with associated elevated alpha feto-protein levels. Repeat biopsy and imaging showed the tumor to be a hepatoblastoma involving both lobes of the liver. The patient was treated by hepatic resection and orthotopic liver transplantation and is doing well at 18 months follow-up.