Objective:the purpose of this review was to evaluate the prevalence of appendiceal endometriosis and the safety of concomitant appendectomy in women with endometriosis or pelvic pain.Materials and Methods:We searched the electronic databases Medline (PubMed), Scopus, Embase, and Web of Science (WOS). The search was not subject to any limitation in terms of time or method. The primary research question was: what is the prevalence of appendiceal endometriosis? The secondary research question was: is it safe to perform appendectomy during surgery for endometriosis? Publications that reported data about appendiceal endometriosis or appendectomy in women with endometriosis were reviewed regarding the inclusion criteria.Results:We found 1418 records. After review and screening, we included 75 studies published between 1975 and 2021. With regard to the first question of the review, we found 65 eligible studies and divided these into the following two categories: (a) endometriosis of the appendix presenting as acute appendicitis, and (b) endometriosis of the appendix as an incidental finding in gynecological surgery. Forty-four case reports described appendiceal endometriosis in women who were admitted for the treatment of pain in the right-sided lower abdomen. Endometriosis of the appendix was observed in 2.67% (range, 0.36–23%) of women who were admitted due to acute appendicitis. In addition, appendiceal endometriosis was an incidental finding during gynecological surgery in 7.23% of cases (range, 1–44.3%). With regard to the second question of the review, which was the safety of appendectomy in women with endometriosis or pelvic pain, we found 11 eligible studies. Reviewed cases had no significant intraoperative or follow-up complications during the 12 weeks.Conclusion:Based on the reviewed studies, coincidental appendectomy appears reasonably safe and was associated with no complications in the cases reviewed for the present report.