We report a case of congenital bilateral pulmonary vein stenosis associated with a double-chambered right ventricle, ventricular septal defect and persistence of the ductus arteriosus in a 29 year-old female. The angiographic, echocardiographic and surgical findings are discussed. A precise diagnosis, the utility of the echocardiography and the surgical correction of this type of ostial stenosis is also reported.