In the last twenty years, there were less than 10 cases of umbilical arteriovenous malformations have been reported, which usually had single complication, included mild cardiac dilatation, pulmonary hypertension, hemorrhagic shock, and hepatic damage. Unlike previous cases, we report a case of neonate multiple organ failure caused by umbilical arteriovenous malformation complicated with portosystemic shunt, which was diagnosed by ultrasound and arteriography. We provide a new insight that umbilical arteriovenous malformation and portosystemic shunt should be considered in neonates with unexplained multiple organ failure, for researching the cause of neonate multiple organ failure, especially in those with right heart overload, hyperammonemia, and liver dysfunction. A targeted and comprehensive Dopler ultrasound can make a definitive diagnosis as soon as possible.