Hilar cholangiocarcinoma (h-CCA) originates from the epithelial cells, which characters as longitudinal growth along the bile ducts and invasion of peripheral vascular nerves. Due to the tumors insidious progression and usually become advanced stage disease at presentation, patients' mortality could parallel incidence rates. For patients who are not amenable to resection, systemic therapy and palliative treatment become the way to go. Dawn of the immunotherapy era offers new opportunity for patients with advanced tumors. Numbers of successful clinical trials have been conducted these years, giving us the chance to optimize multiple treatment modalities. Although liver transplantation is worth to be considered, there is no high-level evidence to support it better outcomes over surgical resection. Given the poor prognosis of h-CCA, radical resection (R0) undoubtfully becomes the only irreplaceable treatment to prolonged survival. Thus, tumors free boundary assessment along the bile duct hit the crucial point. Over the years, numerous imaging techniques leveraging computed tomography, MRI, intraoperation ultrasound and endoscopy with the aim of guiding operation to eliminating cancers. Novel fiberscopes utilizing the second near-infrared region light (NIR-II) offer the potential to assist surgeon visualize tumors precisely. In this review, we summarize the clinical palliative care for advanced h-CCA patients and new opportunities for medications, discussing liver transplantation and other available treatment that not widely disseminated. In addition, we mainly focus on the novel technique of real-time intraoperation imaging navigation to achieve R0 resection and potential molecule prognosis development in the intractable disease.