Abstract: In recent years, the treatment of neuroendocrine neoplasm (NEN) has gained more and more attention; however, few treatment options lead to an objective relief. Somatostatin analogs, everolimus, and sunitinib significantly prolong the progression-free survival (PFS) but can result in a low objective response rate (ORR). The clinical use of streptozotocin-based chemotherapy regimen is limited because of its toxicity and thus is not marketed in China. Some studies with small sample sizes suggest that temozolomide combined with capecitabine (CAPTEM) can result in a higher ORR and longer PFS. However, there is currently no systematic review of the CAPTEM regimen. In this paper, the theoretical basis, efficacy, biomarkers for efficacy predicting, specific drug regimen, and adverse reactions of the CAPTEM regimen for treating neuroendocrine tumors have been reviewed based on studies in recent years.