Objective: To evaluate the ultrasonographic features and pregnancy outcomes in women with ductus venosus-systemic shunt and systematic review of the literature. Method: A computerized search was conducted to identify cases of ductus venosus-systemic shunt between September 2016 and January 2018. Six patients were identified. Antenatal records and neonatal outcomes are presented. A systematic Embase, SCOPUS, and Medline search of published literature from 1991 to 2018 was performed using the terms "ductus venosus," "agenesis," "absence," "absent," "missing," "aberrant," and "variant." Results: Additional structural anomaly such as esophageal atresia with tracheoesophageal fistula was observed in one case. In other case, termination of pregnancy was performed due to Down syndrome. Other two of the six fetuses were monochorionic multiple pregnancies. In our review of the literature, additional anomalies were observed in 9 (42%) of the 21 cases. Conclusion: Our observation suggests that ductus venosus-systemic shunt can be associated with Down syndrome. Detailed examination should be performed to rule out additional abnormalities. Prognosis is good if the pathology is isolated.