Primary pulmonary vein stenosis (PVS) is a rare entity with a high mortality rate. Relatively little is known regarding predictors of outcome or the appropriate timing of intervention. The Pediatric Cardiac Care Consortium database (n = 98,126) was searched for patients who had undergone cardiac catheterization or surgical procedures with primary diagnoses of PVS or atresia from 1982 to 2002. Patients with total or partial anomalous pulmonary venous return, cor triatriatum, previous atrial switch, or previous lung transplantation were excluded. Additional data were obtained through questionnaires sent to each Pediatric Cardiac Care Consortium institution. A total of 31 patients were identified with primary PVS. Excluding lung transplantation, 16 of 31 patients underwent intervention to relieve PVS. Univariate predictors of lung death, defined as death or lung transplantation, included younger age at diagnosis (16.2 vs 52.5 months, p = 0.0221), higher initial mean pulmonary arterial pressure (46.4 vs 26.8 mm Hg, p = 0.0003), and bilateral vessel involvement (lung death in 17 of 19 vs 0 of 9 patients, p <0.0001). Patients diagnosed at 18 months of age and those with initial mean pulmonary arterial pressures >33 mm Hg had incidences of lung death of 76% and 88%, respectively. In conclusion, primary PVS carries a high mortality rate despite attempts at palliation. Patients diagnosed at 18 months of age, having initial mean pulmonary arterial pressures >33 mm Hg, or with bilateral vessel involvement are at significantly increased risk for death or lung transplantation. In these high-risk groups, death occurs rapidly despite intervention, and lung transplantation should be an early consideration.