Background: A superior vena cava aneurym is an extremely rare vascular malformation. They are usually asymptomatic and identified as an incidental finding on chest radiograph. Majority of cases reported are of fusiform type.
Case: This is a case of a cyanotic 5 years old male, who is generally asymptomatic but with failure to thrive. On his first year, he had undergone left cheiloplasty for complete cleft lip and palate. Pre-surgical 2D-echogram revealed Pentalogy of Fallot. There is no family history of congenital heart diseases. Physical examination revealed evidence of chronic hypoxia, pectus carinatum deformity and grade 4/6 systolic ejection murmur. He was followed up on the out- patient department where initial chest radiograph showed a convex soft tissue density in the right anterior mediastinum. This was confirmed to be an aneurysmally dilated superior vena cava measuring 4.4 cm where a horizontally oriented confluence from the left and right pulmonary veins drains.
Conclusion: Giant fusiform Superior Vena Cava Aneurysm in a patient with Supracardiac Total Anomalous Pulmonary Venous Return is an extremely rare occurrence.