Congenital absence of portal vein associated with nodular regenerative hyperplasia of the liver and pulmonary hypertension

We present a 15-year-old girl with congenital absence of the portal vein, pulmonary arterial hypertension and multiple liver lesions proven to be nodular regenerative hyperplasia with biopsy. Ultrasonography, computed tomography, and magnetic resonance imaging findings of the liver lesions and Type I portosystemic shunt are presented.