This report describes the case of a 13-day-old African American female with persistent hypoxia found to have an isolated right superior vena cava (RSVC) draining into the left atrium (LA) confirmed with contrast echocardiography. K E Y W O R D S hypoxia, left atrium, neonate, right superior vena cava How to cite this article: Gulati R, Smith T, Siow M, Batten L. Isolated right superior vena cava drainage into the left atrium: An uncommon cause of persistent hypoxia in a neonate.