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Appendiceal cancer is a very rare disease. Based on 6 cases of appendiceal cancer who were admitted to the Internal Medicine of Breast, Gastroenterology, Hepatology, Urology, Department, Oncology Hospital of Ho Chi Minh City from September 2019 to March 2021, we evaluate the available literature review about diagnosis and treatment of appendiceal cancer. Four out of 6 cases were diagnosed with stage IV disease, only 1 case was in stage II, and 1 case with undermined stage. All cases were treated with Capecitabine-based chemotherapy, 5 out of 6 were treated with Capecitabine and Oxaliplatin combination, while the other was treated with Capecitabine monotherapy. We didn’t use hyperthermic intraperitoneal chemotherapy in any of those cases because of technical limitations at Ho Chi Minh City Oncology Hospital. Up to the time of reporting, all 6 cases are being monitored for survival.