The authors report the rare case of a 12 year old child with an anomalous origin of the left pulmonary artery from the ascending aorta associated with Tetralogy of Fallot. This complex congenital malformation was treated surgically by a Deleval anastanosis between the right subclavian and right pulmonary arteries. The result was good with a 9 year follow-up, the patient having only Grade II effort duspnoea and cyanosis. The authors discuss the advantages and drawbacks of the different possible surgical approaches to this problem in the light of the outcome of this case and a review of the literature.