Early diagnosis is critical in patients with congenital scoliosis to identify vertebral defects that have a high risk for progression. A review of the medical records and chest radiographs for 562 patients who underwent corrective surgery for tetralogy of Fallot (TOF) at our institution between 1992 and 2007 was conducted. Of the 364 patients examined in the study, 12 patients (3.3%), six girls and six boys, had a positive diagnosis of congenital scoliosis confirmed by examination of radiographs. This incidence is significantly higher than both the incidence reported in prior studies (0-1.4%) and in the general population (0.05-0.10%). Given that patients with TOF routinely undergo chest radiograph, physicians examining chest radiographs of TOF patients should be aware of the potential for congenital scoliosis to provide early diagnosis and referral for orthopaedic evaluation and treatment.