Appendiceal mucinous neoplasms constitute a rare pathology, often presenting diagnostic and therapeutic dilemmas due to their heterogeneous presentation and biological behavior. Aim: present a comprehensive review of the existing literature on this topic, our method it is a search in Pubmed using thesaurus vocabulary (MeSH) Medical subjet heading; appendical neoplasm and appendix cancer, abstracts and full text were manually reviewed to identify suitable paper. Conclusion: treatment depends on the stage and type of cancer. Appendiceal neoplasms have the potential to spread within the abdominal cavity and lead to peritoneal carcinomatosis and pseudomyxoma peritonei. Low-grade, initial stage can be successfully managed with surgical removal only, late-stage cancer necessitate cytoreductive surgery and hyperthermic intraperitoneal chemotherapy CRS/HIPEC. Keywords: appendiceal neoplasms, mucinous neoplams, appendix cancer.