This review was written on data collected from case reports, cross-sectional studies, open-label studies and phase –II trials between 1989-2012. Web sites and other online resources of American college of surgeons, Medline, NCBI and Medscape resource centers were used to extract data. Mucocele of the appendix is a rare lesion characterized by the dilatation of the appendix lumen with mucus accumulation. It is more common in 5th and 6th decade with female preponderance. Appendiceal mucoceles can be classified into four histologic groups namely, mucinous cystadenomas, mucosal hyperplasia, simple or retention cysts & Mucinous cystadenocarcinomas. The preoperative diagnosis is usually difficult due to nonspecific clinical presentation. The “onion skin sign” on ultrasound is considered to be specific for mucocele of the appendix. CT scan abdomen usually shows a cystic well-encapslated mass, communicating directly with the cecum sometimes with mural punctate or curvilinear calcification, which may be causing extrinsic pressure on the caecal wall without any surrounding inflammatory reaction. All patients who present with an asymptomatic mass or an incidental cystic lesion of the appendix should undergo surgical exploration, resection and is determined by the organ’s integrity, the dimensions of the base and histological type of the lesion.
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