Background A wide variety of congenital thoracic aortic variants and pathological anomalies could be assessed recently in diagnostic and interventional radiology. Multi-detector computed tomography (MDCT) is one of the most important non-invasive diagnostic tools for their detection. The aim of the study was to evaluate role of MDCT scanning for diagnosis of thoracic aortic anatomic variants and diseases in pediatric patients. Results Thirty patients (15 male and 15 female), mean age (8.49 ± 20.29 months) were diagnosed with different thoracic aortic anomalies by MDCT then confirmed by surgical results. MDCT was more sensitive than echocardiography in detection of hypo plastic arch, vascular rings, interrupted aortic arch anomalies, and aortic coarctation. Both MDCT and echocardiography showed 100% sensitivity in their detection of TGA, TOF, and PDA. MDCT detected 6 cases of right-sided aortic arch while echo missed 2 cases. Different aortic arch branching patterns and coronary origin were better demonstrated by MDCT. Conclusion 320-Multi-detector computed tomography is a reliable tool for optimal detection of thoracic aortic anomalies and preoperative planning.