Objective To validate the applicability of the AJCC 8th edition staging system in predicting prognosis for gallbladder neuroendocrine carcinoma (GB-NEC) and to evaluate the comparative efficacy of different surgical and chemotherapy regimens using data from the Chinese Research Group of Gallbladder Cancer (CRGGC) specialized cohort. Methods Clinicopathological data of 81 patients diagnosed with GB-NEC between 2010 and 2017 across 49 hospitals nationwide were retrospectively analyzed from the CRGGC database. Patient demographics, pathological characteristics, surgical procedures, and chemotherapy regimens were examined. Survival analysis was conducted using the Kaplan-Meier method and Cox proportional hazards regression models. Results The cohort exhibited a male-to-female ratio of 1:1.89. Immunohistochemically, the neuroendocrine markers synaptophysin (Syn), chromogranin A (CgA), and CD56 demonstrated positive rates of 89.2%, 73.8%, and 85.7%, respectively, with Ki67 strong positivity (>50%) observed in 79.7% of cases. Radical resection was achieved in 76.3% of patients. Median overall survival (mOS) was 11.0 (7, 27) months, median progression-free survival (mPFS) was 7.0 (3, 20) months, and the 5-year survival rate was only 13.2%. Univariate analysis revealed that higher AJCC stage correlated with significantly worse prognosis (P<0.001). In patients with TNM stage Ⅲ disease, those undergoing radical surgery had a significantly longer mOS than those receiving non-radical surgery (21.0 months vs. 9.0 months, P=0.041). For patients with advanced disease (stage Ⅲ and Ⅳ), treatment with etoposide plus cisplatin (EP) or irinotecan plus cisplatin (IP) resulted in a significantly superior mOS compared to non-EP/IP regimens (27.0 months vs. 8.0 months, P=0.001). Multivariate analysis identified surgical approach (non-radical vs. radical, HR=2.710, P=0.001), nodal status (N1+N2 vs. N0, HR=2.054, P=0.007), and chemotherapy regimen (non-EP/IP vs. EP/IP, HR=3.576, P=0.001) as independent prognostic factors for GB-NEC. Conclusion The 8th edition AJCC TNM staging system for gallbladder cancer closely correlates with the prognosis of GB-NEC and offers a preliminary reference for clinical prognostic assessment. Radical resection significantly prolongs overall survival in patients with TNM stage Ⅲ disease and should be considered the preferred treatment modality. The EP/IP regimen substantially improves patient survival and represents a potentially optimal chemotherapy strategy. Absence of lymph node metastasis, radical surgical resection, and administration of EP/IP chemotherapy constitute independent predictors of prognosis in GB-NEC.