Cholangiocarcinoma is a lethal disease with increasing incidence worldwide. Perihilar cholangiocarcinoma represents the most common type of cholangiocarcinoma. Despite major development on surgical strategies over the past 20 years, the 5-year survival rate after surgery has remained below 40%, often in the vicinity of 20%. Most perihilar cholangiocarcinomas, however, are unresectable at the time of the diagnosis. The recent use of aggressive approaches based on better image modality, specific perioperative management, and a multidisciplinary approach have enabled to convert the use of palliative therapies to more radical surgery. This review focuses on the recent advances in surgical treatment for perihilar cholangiocarcinoma including liver transplantation with their respective impact on patient survival.