Background and aims: Congenital heart disease (CHD) is described as an abnormality in the heart structure or intra-thoracic great vessels that leads to functional problems. Since most of these disorders require medical and surgical interventions identifying concomitant disorders such as renal and urinary tract abnormalities is of great importance in the management of these patients. The present study aimed to investigate the relative frequency of abnormal kidney and urinary tract findings in abdominal cineangiography during cardiac catheterization of patients with CHD in Shahid Modarres Cardiovascular Medical and Research Center. Methods: The present study was performed prospectively on 545 patients aged < 18 years with CHD who underwent cardiac catheterization and concurrent abdominal cineangiography in Shahid Modarres Cardiovascular, Medical and Research Center, Tehran, Iran during a three-year period. The required data were extracted using a researcher-made questionnaire from patients’ electronic medical files. Results: Of a total of 545 patients in this study, 26 had both CHD and renal or urinary tract malformation. Patent ductus arteriosus was the most common CHD in patients with renal or urinary tract malformations (odds ratio: 1.2, 95%, CI: 2.25–11.63). In this study, the most common renal and urinary malformations among CHD patients was partial duplication of the kidney followed by Ureteropelvic Junction Obstruction.Conclusion: Since the prevalence of renal and urinary tract malformations is higher in CHD patients, performance of concurrent abdominal cineangiography during cardiac catheterization may lead to early diagnosis and treatment as well as better pre- and post-operative management of patients.