A 58-year-old woman underwent surgery for a pelvic mass. Palliative resection was performed despite the presence of multiple retroperitoneal lymph nodes. All pathology specimens exhibited an adenocarcinomatous component associated with carcinoid proliferation related to an appendicular tumor leading to the diagnosis of appendicular adenocarcinoid with ovarian, peritoneal, and nodal metastases. The patient's general status worsened rapidly with widespread nodal metastasis. Chemotherapy (Folfox 4 regimen) was given, and the patient improved within six weeks. Complete response was achieved after three months. Presently, more than three years after the end of the treatment, the patient is still alive and in complete remission. Appendicular adenocarcinoid is exceptional. These tumors exhibit two cellular components. Ovarian metastasis is frequent. Prognosis is intermediate between adenocarcinoma and malignant carcinoid. When given to patients with colonic carcinoma, the Folfox regimen used effectively here, is associated with a 50% objective response but complete response is very exceptional.