Objective To analyze the efficacy and safety of PD-1 inhibitor combined with anlotinib on advanced neuroendocrine carcinoma. Methods We collected the data of patients with advanced neuroendocrine carcinoma who had failed the first-line standard chemotherapy and treated with PD-1 inhibitor combined with anlotinib from the First Affiliated Hospital of Zhengzhou University. Results A total of 45 patients, including 24 males and 21 females, were included. The median age was 57 years old. The primary tumor sites were lung (23 cases, 51.1%), esophagus (8 cases, 17.8%), pancreas (7 cases, 15.6%) and rectum (7 cases, 15.6%). Eighteen cases (40%) had failed the first- and second-line treatments, and 27 cases (60%) had failed the third-line and above treatments. All patients received 2-15 cycles of treatment, 3 cases died due to disease progression, overall objective response rate was 11.1%, disease control rate was 53.5%, median progression-free survival was 5.8 months, and 10-month progression-free survival rate was 25.5%. Adverse events were mainly grade 1-2 myelosuppression and digestive tract reactions. Conclusion PD-1 combined with anlotinib show better efficacy and good tolerance on advanced neuroendocrine carcinoma. It can be used as a choice after the failure of standard first-line treatment of advanced neuroendocrine carcinoma.