Pulmonary veins stenosis in a Down Syndrome infant withnormal connection pulmonary vein is rare and preciseincidence of this disease is unknown. We report a case ofprogressive multiple pulmonary vein stenosis in a DownSyndrome infant with congenital heart disease and transientmyeloproliferative leukaemia. This case-report aims toimprove awareness among physicians and sonographersof this disease and the importance of pulmonary veinassessment in congenital heart disease with unexplainedpulmonary hypertension