Objective To analyze the use of modified pericardial oblique sinus approach in surgical repair for total anomalous pulmonary venous connection in neonates. Methods Between May 2005 and December 2015, 67 consecutive neonates with supracardiac or infracardiac type total anomalous pulmonary venous connection who underwent surgical repair in our institute were included in this study. The patients are divided into three groups according to the different approaches including a sulcus approach group (6 patients), a superior approach group (14 patients), and a modified pericardial oblique sinus approach group (47 patients). There were 53 males and 14 females at median age of 12.5 (7.0, 20.5) d. Results The time of cardiopulmonary bypass [88 (80.0, 107.0) min vs. 135 (121.0, 157.0) min, P<0.05] and aortic cross clamping of the modified pericardial oblique sinus approach group was significantly shorter than that of the sulcus approach group [45 (39.0, 53.0) min vs. 80 (73.0, 85.0) min, P<0.05]. Perioperative mortality (2.1% vs. 28.6%, P<0.05) was significantly lower in the modified pericardial oblique sinus approach group than that in the superior approach group. The long-term mortality (4.3% vs. 60.0%, P<0.05) was significantly lower in the modified pericardial oblique sinus approach group than that in the sulcus approach group or the superior approach group . The rate of pulmonary venous stenosis was significantly lower in the modified pericardial oblique sinus approach group than that in the sulcus approach group (2.1% vs. 50.0%, P<0.05) or superior approach group (2.1% vs. 35.7%, P<0.05). Conclusions In surgical correction of neonatal supracardiac and infracardiac total anomalous pulmonary venous, compared with the traditional surgical approach, the modified pericardial oblique sinus pathway can provide excellent surgical space and has a good surgical prognosis.