This report briefly describes two cases of levoatriocardinal vein, in the setting of mitral atresia and a restrictive interatrial shunt, with clinical as well as echocardiographic features mimicking obstructive total anomalous pulmonary venous connection. One patient died of pulmonary hypertensive crisis, and the other survived the palliative procedures including the atrioseptectomy and pulmonary artery banding. The echocardiography, cine-angiocardiography, and magnetic resonance image for such an anomalous channel are presented. It is tempting to speculate that the thrombocytopenia and indirect hyperbilirubinemia be associated with the destructive consumption of thrombocytes and red blood cells in the serpentine levoatriocardinal vein.