Appendiceal mucinous neoplasm (AMN) is defined as a distended, mucous-filled appendix. It is a rare condition seen in only 8% of cases of appendiceal tumors. Appendiceal mucocele associated with endometriosis is extremely rare with only 16 cases described in the literature to date. In view of the scarcity of the finding, few data are available on preoperative assessment of AMN in women with endometriosis. The goal of the present study was to describe the imaging features that could be suggestive of AMN in the context of endometriosis, as well as the specific management and outcomes.