Congenital portosystemic shunt (CPSS) is a rare vascular abnormality. The number of diagnosed cases is progressively increasing due to the development of imaging features (1,3). It could be found incidentally in patients who are asymptomatic or manifest with hepatic encephalopathy, hypoglycemia, pulmonary arterial hypertension, liver failure, or a liver mass (3), in which case treatment is necessary.