Tetralogy of Fallot’s (TOF) is the commonest cyanotic congenital heart disease with an incidence of 1 in 2000 newborns. Majority of children present in infancy. Left untreated, only 3% survive to adulthood, Anatomically, it consists of 4 components – Ventricular septal defect; Pulmonic stenosis; Right ventricular hypertrophy; Over–riding aorta. TOF with dextrocardia has rarely been reported.

These sporadic case reports have almost all involved cases of mirror–image dextrocardia with situsinversus and TOF. Only one reported case of TOF and dextrocardia with abdominal situssolitus, detected on ultrasonogram in a fetus of 26 weeks’ gestation has been published. The altered spatial orientation of the cardiac structures in case of dextrocardia can present a surgical challenge. A case of TOF with dextrocardia with late presentation during adolescence is presented here.Age at presentation is governed by severity of right ventricular outflow tract obstruction. In the present case, diameter of left pulmonary trunk was 3 mm only. However, right pulmonary trunk had a normal diameter of 10 mm. And this selective obstruction of left pulmonary artery origin is speculated to be the cause of lesser severity of symptoms and, thus, late presentation at the age of 16 years in our case.