Abernethy malformation is a rare condition in which portomesenteric blood bypasses the liver and drains into the systemic vein through a partial or complete shunt. It is categorised into two types on the basis of the shunt pattern between the portal vein and systemic vein. Abernethy malformation is associated with multiple congenital anomalies and acquired complications. A detailed understanding of anatomy and embryology is a prerequisite to interpret the imaging findings. Computed tomography and magnetic resonance angiography can delineate the shunt anatomy and evaluate the concomitant malformations. It is essential to differentiate Abernethy malformation from intrahepatic portosystemic shunts and acquired extrahepatic portosystemic shunts. Mild metabolic abnormalities are treated with dietary modifications and medical therapy. Definitive treatment is done in symptomatic patients. Generally, type I Abernethy patients undergo liver transplantation, and type II undergo shunt occlusion by surgery or transcatheter coiling.