Hilar cholangiocarcinoma (HCCA), a malignant bile duct tumor, has an obscure pathogenesis. We aimed to assess the therapeutic effects of surgery and chemotherapy combined with the PD-1 inhibitor camrelizumab on HCCA patients' prognosis. This study included 26 HCCA patients. Post-surgery, patients received camrelizumab (3mg/kg IV) every 3 weeks, oxaliplatin (100 mg/m2) weekly and gemcitabine (1000 mg/m2) biweekly. Computed tomography assessed treatment response after 6 weeks and survival was evaluated through quarterly follow-ups. Among the 26 patients, 2 achieved complete response, 9 partial response, and 5 stable disease, while 10 progressed. The objective response rate (ORR) and disease control rate (DCR) were 42.3% and 61.5%, respectively. Successful down staging was observed in 16 patients. Post-treatment, AST and ALT levels significantly increased. The overall survival (OS) and disease-free survival (DFS) rates were 76.9% and 69.2%, respectively. Sensitive patients (with down staging) had significantly better survival than non-sensitive patients. Radical surgery and chemotherapy combined with camrelizumab may be effective and safe for HCCA management. Statistical analyses were performed using GraphPad Prism version 7.0, employing Student's t-tests for comparisons between groups.