Purpose Patent ductus venosus (PDV) was a rare congenital portosystemic shunt, and few studies have been reported to date. The purpose of this study was to investigate the clinical and imaging findings to improve the recognition of PDV. Materials and Methods Medical records of 9 patients with PDV in Hunan Children’s Hospital from May 2013 to December 2020 were retrospectively reviewed. The clinical, and laboratory tests data were extracted from the electronic medical records. Two pediatric radiologists evaluated all imaging examinations of the patients.Results 9 patients with PDV were reviewed, including 7 males and 2 females, aged from 16 days to 16.5 years, median age was 1.6 years. PDV diameter ranged from 4.0mm to 17.5mm. The initial clinical presentations of PDV were varied, but jaundice and respiratory symptoms were the most common. Laboratory tests showed that 5/9 cases had hypoxemia, 2/9 had hyperammonemia, 7/9 had hyperbilirubinemia, 6/9 had abnormal coagulation function, 4/9 had abnormal myocardial enzymes, 8/9 had hepatic dysfunction, and 3/9 had renal dysfunction. The direct imaging sign of PDV was a vascular structure connecting the left branch of portal vein(LPV) to the inferior vena cava(IVC), running in the depth of the Arantius sulcus. The secondary imaging findings were as follows: All the patients had enlarged liver, especially the left lobe, and one of the patients presented with diffuse nodules in the liver. 3 patients presented with hypoperfusion in right lobe of liver. The spleen was enlarged in 8 cases but shrank in one. Dilated LPV and atrophic right branch of portal vein (RPV) were observed in all patients. The main portal vein (MPV) was dilated in 8 cases and shrank in one. Dilated right heart and pulmonary artery were observed in all cases. Abnormal renal imaging was observed in 2 patients. The complications and coexistent malformations were as follows: Brain MRI indicated hepatic encephalopathy in 4 cases. 7/9 patients were combined with other malformations, and the most common coexistent malformations were congenital heart disease (CHD) and vascular abnormalities, with 5 and 6 cases respectively. Conclusions PDV was a rare vascular malformation that can lead to multi-system lesions. The clinical presentations and laboratory findings were diverse. The diagnosis of PDV mainly depends on imaging examinations, and it is important to evaluate the secondary imaging changes. Complications and coexistent malformations were common, and we need to prevent omissions during the imaging evaluations.