Goblet cell carcinoid (GCC) of the appendix is characterized by a dual differentiation of both endocrine and gland. This study, therefore, explores its clinicopathological and immunohistochemical features. The authors reviewed clinicopathological and immunohistochemical features of 26 GCC cases in Southwest China between 1991 and 2009. The incidence is 0.0453%, with a gender ratio of 2.71:1 (19 males and 7 females). Three cases were combined with schistosomiasis, suggesting schistosomiasis may be associated with GCC. Eighteen cases available for immunohistochemistry showed a few scattered positive cells for CD56, NSE, CgA, and Syn; diffuse positive for CEA; and preserved positive for E-cadherin and β-catenin. Follow-up data showed that 1 patient died from peritoneal metastasis and that the other 17 cases survived free of the tumor. The authors characterize GCC as a special low-grade malignant carcinoma with a primary epithelial differentiation and little neuroendocrine differentiation. A combination of both histological features and immunoreactivity is needed to diagnose GCC.