Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 10 1 2025 09 08 7 11 Maziar Karamnejad Research Center for Advanced Technologies in Cardiovascular Medicine, Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran. Mahdis Nazari Zanjan Metabolic Disease Research Center, Health and Metabolic Disease Research Institute, Zanjan University of Medical Sciences, Zanjan, Iran. Ehsan Parvas Research Center for Advanced Technologies in Cardiovascular Medicine, Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran. Reza Elahi Department of Radiology, Zanjan University of Medical Sciences, Zanjan, Iran. Amir Jolfayi Cardiovascular Research Center, Rajaie Cardiovascular, Medical, and Research Center, Iran University of Medical Sciences, Tehran, Iran. Kyomars Abbasi Research Center for Advanced Technologies in Cardiovascular Medicine, Tehran Heart Center, Tehran University of Medical Sciences, Tehran, Iran. 2025 04 29 2025 07 20 Scimitar syndrome is a rare congenital cardiac anomaly characterized by abnormal drainage of the right pulmonary veins into the inferior vena cava (IVC). Here, we report the case of a 26-year-old female diagnosed with scimitar syndrome (SS), with a history of imperforate anus and vaginal agenesis, consistent with a VACTERL association. The imperforate anus was identified and treated in infancy, whereas the vaginal agenesis was diagnosed at menarche. Additionally, the patient presented with a large atrial septal defect (ASD) and a hypoplastic right lung alongside SS. This case underscores the potential correlation between SS, vaginal agenesis, and imperforate anus as features within the VACTERL spectrum. https://crcp.tums.ac.ir/index.php/crcp/article/view/1043 https://crcp.tums.ac.ir/index.php/crcp/article/download/1043/704