We present the case of a 24-year-old man who was incidentally diagnosed with congenital extrahepatic portosystemic shunt with portal vein aneurysm during an investigation for non-specific abdominal pain. These are rare anomalies, and to the best of our knowledge, this is the first case reported with both anomalies associated together. Ultrasound, including color Doppler, computed tomography, and magnetic resonance imaging were performed which revealed a side-to-side shunt between the extrahepatic portal vein and the inferior vena cava, with aneurysmal fusiform dilatation of the proximal intrahepatic portal vein which ended abruptly. Etiology, clinical significance, and management strategies with regard to these abnormalities are discussed.

Keywords: Abernethy malformation • portal vein aneurysm • computed tomography