Tetralogy of Fallot with anomalous pulmonary venous connections: a rare but clinically important association. Anomalous pulmonary venous connections were found in seven (0.6%) of 1183 patients with tetralogy of Fallot. Three patients had totally anomalous connections (one supracardiac, one direct to coronary sinus, and one mixed supracardiac and infracardiac) and four patients had partially anomalous pulmonary venous connections. All patients presented with the clinical features of tetralogy of Fallot. Anomalous pulmonary venous drainage was suspected clinically in only one patient in whom there was a scimitar sign on the chest radiograph. The exact diagnosis was established by cross sectional echocardiography (one), preoperative or postoperative angiography (five), or at necropsy (one). Surgery was performed in six patients. Total correction without re-routing of the anomalously draining veins was successful in all those with partially anomalous connections, with no significant long term sequelae (follow up median 17 years). Of those with totally anomalous connections, the full diagnosis was made only at necropsy in one patient, successful one-stage correction was performed in one, and the other patient, who had partially obstructed mixed drainage, died shortly after one-stage correction. Histological examination of the lung biopsy specimen in this patient showed grade 2 pulmonary vascular disease. Tetralogy of Fallot with anomalous pulmonary venous connections is a rare association. Careful preoperative assessment is required in those with totally anomalous connections. 