The purpose of this study is to evaluate recently reported outcomes for treatment options for proximal cholangiocarcinoma (CCA). Standard evidence based practice techniques were used to formulate a question, search, appraise and evaluate the retrieved literature. Our question was "In patients with CCA, how do stenting alone, stenting in addition to brachytherapy (BT) or photodynamic therapy (PDT), resection and orthotopic liver transplantation with neoadjuvant chemoradiation (OLT) compare for long-term survival? Level 1b survival data was available for stenting alone (179 days), BT and metal stenting (388 days) and PDT with plastic stenting (493 days) and no survival difference was evident with metal vs. plastic stenting or unilateral vs. bilateral stenting. Five year survival data (level 3) was available for OLT (80%), formally curative trisegmentectomy with or without portal vein resection (72% and 52%) and hepatectomy (18%-23%). All patients with proximal CCA should be reviewed by a multidisciplinary team to determine appropriate treatment. For unresectable CCA, patients should be assessed for OLT with neoadjuvant chemoradiation, while those who are unsuitable would appear to have the longest survival with PDT. Extended resection in operable candidates may improve survival over right or left hepatectomy but increased perioperative mortality is a consideration.