A congenital portosystemic shunt of the portal vein is a very rare vascular anomaly associated with the liver. We report the case of a 5-year-old girl with a patent ductus venosus and her 31-year-old mother with a congenital portosystemic shunt. The child presented with a history of an extremely low birth weight in addition to an atrial septal defect and a patent ductus venosus. At the age of 2, she underwent ligation of the ductus venosus. Her mother was also diagnosed with a congenital vascular anomaly at the age of 16. We have followed up and evaluated her asymptomatic mother for 15 years. To our knowledge, this is the first report describing the occurrence of a congenital portosystemic shunt in both a mother and her child.