We report a 10 year old girl who was detected to have a sinus venosus a trial septal defect with complex partial pulmonary venous connections. Left pulmonary veins were draining into right superior vena cava via the innominate and vertical vein, and right upper pulmonary vein was draining directly into superior vena cava. Only the right lower pulmonary vein was connected to the left atrium. Though any combinations of pulmonary venous drainage are reported in literature, current reported type of pulmonary venous connections is one of the rarest type with only few cases previously reported. Through this report we also want to emphasize the need for screening for all pulmonary veins every case of atrial septal defect especially in sinus venosus type defect and when there is discrepancy between size of atrial septal defect and haemodynamic disturbance.
Key words: Atrial septal defect, Pulmonary venous connections, Sinus venosus.