Background <p>Cor triatriatum dexter (CTD) is a rare congenital cardiac anomaly resulting from persistence of the right valve of the sinus venosus. Clinical presentation varies from neonatal hypoxemia to incidental diagnosis in adulthood. Optimal management strategies remain poorly defined due to limited published literature. </p> Methods <p>We report a single-center series of four patients diagnosed with CTD. Clinical presentation, echocardiographic findings, surgical management, and postoperative outcomes were retrospectively reviewed.</p> Results <p>All four patients presented in the neonatal period with hypoxemia and failed critical congenital heart disease (CCHD) pulse oximetry screening, ultimately resulting in echocardiographic diagnosis of a redundant membrane within the right atrium consistent with CTD. All four patients underwent surgical repair at an average of 21 days-of-life (5–41 DOL) and 4 kg (3.4–4.5 kg). Mean cardiopulmonary bypass time was 33 minutes (14–44 min). Mean postoperative length of stay was 6 days (3–9 days). Follow-up was available in 3 patients, all of whom were doing well with no cardiac symptoms and on no cardiac medications up to 7 years postoperatively.</p> Conclusions <p>CTD repair can be performed with a relatively short cardiopulmonary bypass time, limited postoperative hospital stay, and no cardiac concerns at medium term follow-up. We advocate for an individualized approach when deciding surgical intervention vs. watchful waiting but generally err on the side of early surgical repair.</p>