Biliary atresia is a devastating fibro-obliterative cholangiopathy of infancy. Currently, the interventions that can alter the course of afflicted infants are Kasai Portoenterostomy (KPE) and liver transplantation. Timely KPE is critical, increasing the likelihood of durable biliary drainage. Some patients present with advanced liver disease that is unlikely to respond to KPE while others fail to drain after KPE, both of which then require liver transplantation to survive. Given the significant improvement in outcomes in pediatric liver transplantation, some have proposed that patients with BA should undergo liver transplantation primarily, sparing the morbidity and increased technical complexity of liver transplantation after KPE (salvage transplant). In this chapter, we reviewed the existing literature comparing outcomes of KPE versus primary liver transplantation and primary versus salvage liver transplantation. We found that outcomes after salvage liver transplantation are similar to primary liver transplantation and because successful KPE precludes the risks of transplantation (peri-operative transplantation morbidity, mortality, and complications from long-term immunosuppression (infection, calcineurin induced hypertension, chronic kidney disease, and post-transplant lymphoproliferative disease)), we recommend that KPE be the first treatment offered. Despite this, there is a high likelihood that in almost all patients afflicted with BA, liver transplantation will be necessary. A prospective study is needed to identify patients who will not respond to KPE who should then undergo primary liver transplantation.