Abstract: Objective: This article aimed to analyze the computed Tomography (CT) imaging manifestations and clinical significance of scimitar syndrome to improve our understanding of the disease. Method: A total of 28 patients diagnosed with scimitar syndrome were enrolled in our hospital from January 2019 to January 2024, and their clinical and imaging data were retrospectively analyzed. Results: Among the 28 cases, there were 10 males and 18 females, with 15 and 13 cases of the infantile and adult types, respectively. The average age of patients with the infant type were (6.78±3.50) months, with 10 cases having total anomalous right pulmonary venous drainage. Among them, three cases had stenosis and obstruction at the site of ectopic drainage and two cases had concomitant atrial septal defect and pulmonary arterial hypertension; these five cases underwent surgical treatment. Five cases of partial anomalous right pulmonary venous drainage to the inferior vena cava were found during imaging examination owing to the presence of other deformities and pulmonary infections. The average age of patients with the adult type was (5.53±2.72) years old. Seven cases had total anomalous right pulmonary venous drainage to the inferior vena cava, with one case undergoing surgical treatment. Six cases had ectopic drainage from the right lower pulmonary vein to the inferior vena cava: two underwent elective surgery owing to a clear diagnosis for many years, and two underwent interventional treatment for systemic arterial blood supply owing to extralobar sequestration. Conclusion: There were significant differences in clinical manifestation between patients with infantile and adult types of scimitar syndrome. Using multiple imaging modes of CT examination combined with powerful reconstruction post-processing functions, children with scimitar syndrome can be evaluated comprehensively, providing a basis for the selection of clinical treatment methods and surgical plans.