A case of a 53-year-old male with a long-lasting pulmonary hypertension of an unknown cause is presented. Both transthoracic and transesophageal echocardiography as well as cardiac catheterisation failed to document any cardiac defect. Finally, a multi-slice spiral computerised tomography revealed the presence of an anomalous pulmonary vein drainage to the superior vena cava with concomitant inter-atrial septal defect of a sinus venosus type. The patient was selected for surgery.