Unilateral pulmonary vein atresia is a rare condition in adults that may cause serious diagnostic problems because of unspecific clinical findings. A 28-year old female patient is described who presented with signs of recurrent pulmonary embolism with shortness of breathing, unilateral thoracic pain and several episodes of haemoptysis. Ventilation and perfusion scans showed a total lack of perfusion in the right lung with only slight disturbance of ventilation. However, no marked increase of pulmonary artery pressure and no signs of a recent thrombosis of peripheral veins were found. Transoesophageal echocardiography and pulmonary angiography in combination with aortography revealed the diagnosis of unilateral pulmonary vein atresia with abnormal branches of bronchial arteries. There is a considerably left-to-right shunt with return of flow from the bronchial arteries to the right and afterwards to the left pulmonary artery. Clinical, radiological and nuclear medical findings as well as therapeutical options are described.