Background/Aims: Perihilar cholangiocarcinoma is a rare disease with unfavorable prognosis resulting in low survival rates. This studyaims to retrospectively assess the beneficial histopathological features and surgical procedures in long-term survivors (i.e., patientssurviving perihilar cholangiocarcinoma for at least 2 y).Material and Methods: In total, 322 patients with perihilar cholangiocarcinoma underwent surgery at our center. The follow-up endedin 2017; 76 patients survived for >2 y. The type of resection, UICC stage, and histopathological features were compared between threesurvival groups (>2-3, >3-5, and >5 y).Results: The >5-year-survival rate in our selected study cohort was 43.4% (>3-5 y,31.6% and >2-3 y, 25.0%), and 14.5% of the patientssurvived for >10 y after surgery. Patients with non-regional lymph node positive tumors and/or distant metastasis (i.e., UICC stage IVb;p=0.0112), R2 status (p=0.0288), and exploratory laparotomy only (p=0.0157) showed the poorest survival rates. Perineural invasionhad no significant impact on the overall survival. However, 29.0% patients surviving for >5 y displayed the lowest perineural infiltrationprevalence. Interestingly, Bismuth-Corlette stage IIIa (p=0.0467), especially caudate lobectomy (p=0.0034), was associated with disease-specific overall survival of >5y.Conclusion: Complete/extended tumor resection with additional caudate lobe resection is strongly associated with long-term survival.Perineural infiltration as a negative prognostic marker for prolonged survival needs to be evaluated in larger study cohorts.