The authors present the case of a 14-year-old boy with secondary portal hypertension caused by arterio-portal fistulae. Ascites, edema, severe abdominal pain, and steatorrhea developed in the patient. In an attempt to decrease arterioportal flow the authors carried out vascular embolization that was partially successful. A liver transplant was performed as a last therapeutic resource in view of the fact that it constitutes an accepted therapy for patients with severe liver disease. Orthotopic liver transplantation may be considered as another possible treatment of arterio-portal fistulae.