A 52-year-old patient presented with epigastric pain.An enhanced CT scan showed a strongly enhanced appendix with abscess formation.Appendectomy was performed under the diagnosis of acute appendicitis with perityphlitic abscess.The stump of the appendix was white and hard, suggesting malignant transformation.Intraoperative frozen sectional examination indicated goblet cell carcinoid(GCC)of the appendix.Thereafter, we performed ileocecal resection with lymphadenectomy (D3).The final pathological diagnosis was GCC, pSS, pN1, Stage III a by the Japanese classification of colorectal carcinoma. Immunohistochemical examination was consistent with GCC including synaptophysin(+), chromogranin A(+), somatostatin receptor(SSTR)2(±), SSTR5(+), and cytokeratin 20(+).The patient received adjuvant chemotherapy and remains cancer-free over 5 years after the operation.