Introduction. Gastrointestinal stromal tumors (GISTs) are the most frequent mesenchymal neoplasms of the gastrointestinal (GI) tract. However, rectal GISTs are rare among these tumors. Case Presentation. A 60-year-old Caucasian woman had surgery for an ovarian mass with carcinomatosis. The patient underwent exploratory laparoscopy, which found a large amount of ascites, epiploic and appendicular carcinomatosis. The Hudson operation was performed to completely and en bloc remove the recto-sigmoid junction, the uterus, and adnexa. The appendix and the carcinomatosis were also removed with no residual tumor. After that, she was sent to the oncology department to start Imatinib therapy. Conclusion. Rectal GIST is a rare entity with varied clinical manifestations and a high risk of recurrence, which may complicate the diagnosis in women with an abdominopelvic tumor mass. Consequently, even if GIST can mimic gynecological tumors, such as ovarian tumor, the surgeon must be aware of this condition and take it into account in establishing the differential diagnosis, especially since surgical treatment is essential in localized rectal GIST.
