Right atrial isomerism is well known to be associated with anomalous pulmonary venous drainage and a potentially dangerous sequel to pulmonary venous obstruction, and is associated with high mortality. We report a 16-month-old girl with pulmonary venous obstruction requiring lobectomy for hemoptysis after a Glenn operation. Two years after surgery, she is doing well with 80% oxygen saturation and no further episodes of hemoptysis.