Although there are reports of goblet cell carcinoid(GCC)treated by chemotherapy using the treatment protocol for colon cancer, the benefit of chemotherapy for GCC remains controversial and unclear. Herein we report 2 cases of patients with GCC who were successfully treated by surgical resection and oxaliplatin-based adjuvant chemotherapy, without evidence of recurrence. The first case was a 57-year-old man who underwent laparoscopic ileocecal resection after being diagnosed with adenocarcinoma of the appendix by biopsy via colonoscopy. Pathological and immunohistochemical analyses demonstrated the presence of signet-ring-like cells, chromogranin A-positive and synaptophysin-positive cells, leading to a diagnosis of GCC of the appendix. Folinic acid, fluorouracil, oxaliplatin(FOLFOX)was administered for 6 months as an adjuvant chemotherapy. The patient has shown no signs of systematic metastasis and has been alive for more than 3 years after the operation. The second case was a 41-year-old woman who presented to our hospital complaining of lower abdominal pain starting 2 months previously. A computed tomography(CT)scan indicated a pelvic tumor, and she underwent ileocecal resection and hystero-oophorectomy due to an appendix tumor and an ovarian metastatic tumor. Pathological and immunohistochemical analyses demonstrated the presence of signet-ring-like cells, chromogranin A-positive, and synaptophysin-positive cells, confirming the diagnosis of GCC of the appendix. The patient received capecitabine and oxaliplatin(CapeOX)as an adjuvant chemotherapy for 6 months. The patient has been free from recurrence for 22 months following surgery.