Common pulmonary vein atresia is a very rare from of congenital cardiac anomaly in which the common chamber of pulmonary veins has no direct communication to the heart or systemic venous system. We report here on one-day-old boy with total anomalous pulmonary venous connection having severe stenosis (sized less than one millimeter in diameter) of vertical vein between common pulmonary vein and superior vena cava. His anatomic feature was considered as a spectrum between common pulmonary vein atresia and total anomalous pulmonary venous connection because of almost atretic draining vertical vein. His clinical presentation and hemodynamic characteristics were the same as common pulmonary vein atresia. He underwent an emergent anastomosis of common pulmonary vein to left atrium at 33 hours after birth under cardiopulmonary bypass, cross-sectional echocardiography and Doppler color mapping. He was discharged home two months after the surgery. Cardiac catheterization after six months revealed normal cardiac function without any evidence of pulmonary venous chamber stenosis. To improve the outcome of surgical intervention for this life-threatening cardiac anomaly, immediate the precise diagnosis using echocardiography followed by emergent surgical correction is in dispensable.