In a 50-year-old male with an incidentally discovered hepatocellular carcinoma, a congenital extrahepatic portosystemic shunt with the absence of the portal vein (Abernethy syndrome) was diagnosed by imaging. This study aims to discuss the variant anatomy relevant to this splanchnic vascular malformation, review its association with the development of liver neoplasms, and report the safety and efficacy of TARE followed by resection for a HCC arising in this rare condition.
Keywords: Abernethy malformation; Congenital extrahepatic shunt; Hepatocellular carcinoma; Radioembolization.