Patients with major aortopulmonary collateral arteries usually present in one of three ways: either with marked heart failure because of lung overflow, cyanotic because of reduced lung flow, or fairly well balanced with systemic oxygen saturations in the high 70s to low 80s. All patients require a planned cardiologic surgical approach, with careful investigation to delineate the collateral morphology. A carefully coordinated, combined approach between surgery and cardiology intervention is required throughout the treatment of these patients. The majority of these patients now enter a program of reconstruction of the collaterals to a valved right ventricular pulmonary artery conduit with or without ventricular septal defect closure. Further catheter intervention to stretch and enlarge the pulmonary arteries may be necessary, followed by staged ventricular septal defect closure. Other techniques to enlarge central pulmonary arteries or to recruit collaterals can be used. Outcomes over the last 20 years have been satisfactory, with survival of 80% over 10 years, which is a marked improvement on the natural survival in this group of patients.