We describe a patient with liver scarring associated with congenital absence of the right lobe of the liver. Computed tomography demonstrated a low density zone at the periphery of the liver that appeared hyperintense on T2-weighted MRI and markedly enhanced on contrast-enhanced T1-weighted imaging. Computed tomography during both arteriography and arterial portography demonstrated a differential blood supply to the low density zone, suggesting the diagnosis of liver scarring. Histological examination of a biopsy from this region disclosed periportal fibrosis and sinusoidal dilatation, confirming the diagnosis of liver scarring.