Pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals are a challenging congenital anomaly to manage surgically with different centers and strategies producing wide-ranging outcomes. Over the past few decades despite diverging treatment pathways, there is an emerging consensus of how these patients "should" be treated. Quite often a combination of rehabilitation strategy and a unifocalization approach has to be tailored to each patient to address the anatomic and physiological variations that characterize this congenital heart defect. Irrespective of the surgical approach, the goal should be to have a complete repair with acceptable right heart pressure ensuring survival and a good quality of life.