Purpose of reviewAbnormal appendiceal disease is commonly encountered following an appendectomy when performed in patients with endometriosis. Appendiceal endometriosis is the most notable finding and can affect up to 39% of patients with endometriosis. Despite this knowledge, guidelines for performing an appendectomy have not been formally established. In this article, we review the surgical indications for an appendectomy at the time of endometriosis surgery and discuss the management of other diseases that may be encountered following the histopathologic evaluation of an excised appendix.Recent findingsRemoval of the appendix in patients with endometriosis contributes to optimal surgical management. Relying on abnormal appendiceal appearance for removal may leave endometriosis-affected appendices. For this reason, utilizing risk factors to guide surgical management is essential. Common appendiceal diseases are sufficiently managed with appendectomy. Uncommon diseases may require further surveillance.SummaryEmerging data in our field support the performance of an appendectomy at the time of endometriosis surgery. Guidelines for performing a concurrent appendectomy should be formalized to encourage preoperative counselling and management for patients with risk factors for appendiceal endometriosis. Abnormal diseases is frequently encountered after appendectomy in the setting of endometriosis surgery and further management is based on the histopathology of the specimen.