The congenital extrahepatic portosystemic shunt is a rare anomaly characterized by aplasia or hypoplasia of intrahepatic portal venous branches that generate complete or partial extrahepatic shunting of portal venous intra-systemic veins. The clinical presentation is variable, including nausea, abdominal pain, anorexia, and jaundice. This entity has been associated with other congenital anomalies, and the most common are the cardiovascular anomalies. Imaging studies are essential to confirm the diagnosis, establish pre-operative planning, and determine the surgical procedure to be performed. We presented the case of a 15-year-old adolescent who had abdominal symptoms and was found to have a portal venous vascular abnormality.