Background: An abnormal coronary artery crossing the right ventricle outflow tract (RVOT) complicates complete repair of tetralogy of Fallot (TOF). We have evaluated surgical options for RVOT reconstruction for this group of patients. Methods: Between 1984 and 2002, 35 TOF patients with abnormality of coronary arteries underwent total correction. Age of these patients ranged from 1 to 14 years (mean 5.8 +/- 2.8 years). All patients were diagnosed by echocardiography and 25 patients had cardiac catheterization. All the abnormal coronary arteries were spared at the operation. In 24 patients a transannular patch was applied for RVOT reconstruction. In three patients with an adequate pulmonary annulus, oblique ventriculotomy incisions, and in two patients, transatrial approaches were performed. "Two-patch" technique was chosen for two patients. In four patients placement of an extracardiac conduit was necessary. Results: Mortality was observed in four patients, in two of them due to suspected myocardial ischemia. None of the surviving patients needed reoperation. All of them were in NYHA class I. The follow-up period for patients without extracardiac conduit was between 14 and 96 months (mean 50.2 months) and for the patients with extracardiac conduit ranged 36 to 98 months (63.5 months). The mean gradients measured by echocardiography were, respectively, 5 mmHg (range 0 to 35 mmHg) and 23.75 mmHg (range 20 to 25 mmHg). Conclusions: Definitive repair of TOF patients with abnormal coronary arteries can be performed in early childhood, but care should be taken to leave at least 1 cm of myocardium between the sutureline and the abnormal coronary artery. Detailed evaluation of the patients preoperatively is mandatory to identify the strategy and timing of the operation.