Congenital absence of the portal vein is a rare clinical entity in which portal blood flow is partially or totally diverted to the systemic circulation.This condition causes a myriad of clinical symptoms and is associated with other congenital abnormalities mostly of cardiac and vascular origin.A 14-year-old male patient with type IB Abernethy malformation who presented with macroscopic hematuria was treated with live donor livertransplantation. The medical recordings of the patient were retrospectively reviewed, and the case was presented with a brief review of the literature.The patient had associating nut-cracker syndrome and liver masses that were revealed to be focal nodular hyperplasia. The patient is doing well inthe postoperative course. Liver transplantation offers a safe and radical treatment for this very rare anatomic malformation. It not only correctsaberrant portal flow but also prevents other life-threatening complications such as hepatopulmonary syndrome and possible liver malignancies.