Objective: Studies on lung metastases of extrahepatic cholangiocarcinoma (ECC) are rare. This study aims to fill this gap by analyzing the influencing factors, prognosis, and treatment effects of ECC lung metastases, and to provide clinical guidance for their diagnosis and treatment. Methods: We retrieved data from the Surveillance, Epidemiology and End Results (SEER) database for patients with metastatic ECC (stage M1) from 2018 to 2021. The study analysed these characteristics using descriptive statistics. To calculate hazard ratios, multivariate COX regression analyses were performed. Overall survival (OS) was estimated using the Kaplan-Meyer method, and the survival of patients between groups was compared using the log-rank test. Results: A total of 762 people participated in the study, 50.4 per cent of whom were men. At the time of diagnosis, 17.8% of patients had pulmonary metastases. 52.5% received chemotherapy. Multivariate COX analysis identified lung metastases as a significant risk factor for death from metastatic ECC (HR 1.64, CI 1.32-2.03, P<0.001). Chemotherapy (HR 0.20, CI 0.17-0.25, P<0.001) and female (HR 0.80, CI 0.67-0.94, P=0.008) were associated with a better prognosis. Therefore, we further compared the prognosis and chemotherapy outcomes of male and female patients with ECC lung metastases. The median survival of male patients with and without lung metastases was 2 and 5 months, respectively (P=0.016), whereas there was no significant difference in female patients (P=0.19). Regardless of gender, patients with lung metastases had significantly worse OS even after receiving chemotherapy (P=0.0065 in the male group and P=0.0075 in the female group). Regardless of gender, patients with lung metastases who did not receive chemotherapy had significantly shorter overall survival than those who received chemotherapy. Not receiving chemotherapy vs receiving chemotherapy (male: 1 month vs 5 months, P<0.0001; female: 2 months vs 9 months, P<0.0001). Conclusion: Pulmonary metastases are an important prognostic factor in ECC and are associated with poorer survival, especially in male patients. In all patients with metastatic ECC, chemotherapy is associated with prolonged survival. Therefore, preventive measures and effective control of pulmonary metastases (such as chemotherapy), especially in male patients, may improve survival in patients with ECC.