Congenital absence, or hypoplasia, of the superior vena cava (SVC) with situs solitus is quite uncommon. Most cases have been found incidentally and concomitant with left persistent SVC or other cardiac disorders. Congenital chylothorax may be associated with cardiac anomalies, various syndromes, thoracic ductal or venous thrombosis, birth trauma or local compression of the thoracic duct, but it is rarely caused directly by congenital SVC anomalies. Conclusion: We report a rare case of congenital chylothorax associated with isolated congenital hypoplastic SVC and underdeveloped collateral circulation. Congenital absence, or hypoplasia, of the superior vena cava (SVC) with situs solitus is quite uncommon. Most cases have been found incidentally and concomitant with left persistent SVC or other cardiac disorders. Congenital chylothorax may be associated with cardiac anomalies, various syndromes, thoracic ductal or venous thrombosis, birth trauma or local compression of the thoracic duct, but it is rarely caused directly by congenital SVC anomalies. Conclusion: We report a rare case of congenital chylothorax associated with isolated congenital hypoplastic SVC and underdeveloped collateral circulation.