Cor triatriatum (CTT) sinistrum is a rare, congenital left ventricular inflow anomaly. Previous reports have used cardiac angiography, two-dimensional echocardiography, transesophageal echocardiography and magnetic resonance imaging to establish the diagnosis prior to surgery. We report a case of CTT diagnosed by echocardiography whose hemodynamic significance was established by the use of pulsed wave and color Doppler techniques. Immediate surgery was recommended based on anatomical and hemodynamic parameters.