Background and objectives: Primary pulmonary vein stenosis or atresia (PVS/A) is a rare entity with a very high mortality rate. The aim of this study was to elucidate clinical feature and prognostic factors of primary PVS/A in children. Methods: We reviewed the medical records of pediatric patients who had diagnosed with primary PVS/A with anatomically normal pulmonary vein (PV) connection at Seoul National University Children���s Hospital from 1999 to 2009. Results: Fifteen cases were identified. The median age at initial presentation was 15 months. Major symptoms were hemoptysis (53%) and dyspnea (40%). Eleven had associated cardiac lesions; 5 of them had history of cardiac surgery unrelated to PV before the occurrence of pulmonary vein stenosis (PVS). The median number of initially involved PVs were 2 (range: 1-4) and 27% showed bilateral involvement. During the following period (mean 9 months; range 0.2-131 months), PVS progressed to additional veins in 7 cases. Thirteen patients (86%) underwent surgical intervention (venoplasty in 8 and pneumonectomy in 3 patients). All the patients who underwent venoplasty had restenosis after few months (median 2months). Sutureless technique did not reduce the restenosis rate. Three patients had functional single ventricle, and two Fontan patients survived without symptom after pneumonectomy or venoplasty. Mortality rate was 46.7%. Median age of death was 10.8 months and the median time between diagnosis and death was 3 months. Univariate predictors of death included multiple stenosis (���3 veins) or bilateral involvement (p=0.001, OR=8, 95% CI 1.28~50.04) and progressive PVS (p=0.01, OR 42, 95% CI 2.14-825.72). Conclusion: Primary pulmonary vein stenosis or atresia had significant risk of recurrent and progressive obstruction or death even after surgical venoplasty. All bilateral and multiple PVSs were fatal.