The coexistence of cor triatriatum and tetralogy of Fallot (TOF) is rare. Preoperative identification of cor triatriatum may be difficult owing to reduced pulmonary blood flow in patients with TOF. However, it is imperative to identify this rare combination as failure to identify obstruction to pulmonary venous egress may result in persistent pulmonary venous hypertension postoperatively. The authors discuss hemodynamic aspects of this rare coexistence in a 14-month-old child in whom pulmonary capillary wedge pressure was elevated despite right ventricular outflow obstruction. © 2012 Wiley Periodicals, Inc.