Primary adenocarcinoma of the appendix is a rare ma-lignancy that constitutes < 0.5% of all gastrointestinal neoplasms. Moreover, primary signet ring cell carci-noma of the appendix is an exceedingly rare entity. We have encountered 15 cases of primary appendiceal cancer among 3389 patients who underwent appen-dectomy over the past 18 years. In the present report, we describe a rare case of primary signet ring cell carcinoma of the appendix with ovarian metastases and unresectable peritoneal dissemination occurring in a 67-year-old female patient. She underwent ap-pendectomy and bilateral salpingo-oophorectomy with a laparoscopy procedure. She then received palliative systemic chemotherapy with 12 cycles of oxaliplatin, 5-flurorouracil, and leucovorin (FOLFOX-4). The patient currently is well without progression of disease 12 mo after beginning chemotherapy. Primary adenocarcinoma of the appendix is ​​a rare ma-lignancy that that <0.5% of all gastrointestinal neoplasms. Moreover, primary signet ring cell carci-noma of the appendix is ​​an exceedingly rare entity. We have encountered 15 cases of primary appendiceal cancer among 3389 patients who underwent appen-dectomy over the past 18 years. In the present report, we describe a rare case of primary signet ring cell carcinoma of the appendix with ovarian metastases and unresectable peritoneal dissemination occurring in a 67-year-old female patient. She underwent ap-pendectomy and bilateral salpingo-oophorectomy with a laparoscopy procedure. She then received palliative systemic chemotherapy with 12 cycles of oxaliplatin, 5-flurorouracil, and leucovorin (FOLFOX-4). The patient currently is well without progression of disease 12 mo after beginning chemotherapy.