ObjectiveTo report a rare, misleading fibroid degeneration involving a non-functional, non-communicating horn in a woman with a unicornuate uterus. While the presence of a functional rudimentary horn may lead to signs and symptoms that recommend its removal, non-functional cases are rarely reported, and due to their apparent functional inactivity, the need for their removal has never been reported. No previous report showed the possibility of a degenerative process in a non-functional rudimentary horn causing patient discomfort. DesignThis is a step-by-step narrated video showing a unique case of fibroid degeneration and growth of a non-functional, non-communicating rudimentary horn in a unicornuate uterus (ASRM classification 2021) and its surgical management. SettingUniversity Academic Hospital ‘‘A.O.U. delle Marche – Università Politecnica delle Marche” Ancona, Italy. SubjectA 48-year-old Caucasian nulliparous premenopausal woman was referred to our institution because of abdominal pain and an enlarging adnexal mass. Her personal history showed primary infertility with a previous diagnosis of unicornuate uterus. Given the possibility of ectopic ureters in these occurrences, a complete computed tomography scan was performed, and no genitourinary alterations were found. Pre-operative imaging (ultrasound evaluation, computed tomography, and magnetic resonance) provided a provisional diagnosis of a suspicious ovarian fibroma. InterventionsConsidering the patient’s age, lack of desire for pregnancy, and volumetric increase of the adnexal mass, a laparoscopic intervention to perform mass removal and prophylactic bilateral salpingectomy was planned. The patient was counseled about the low risk of an underlying malignant transformation. Therefore, the decision to remove the intact mass via a mini-laparotomy at the end of the surgery was shared.Once the abdominal cavity was entered, the right unicornuate uterus was found in anatomical continuity with the ipsilateral broad ligament, fallopian tube, and ovary. These structures were wholly attached to the right pelvic wall.On the other side, cranially compared to the right hemi-uterus, a roundish myoma-like mass was detected in direct connection with the left broad ligament, fallopian tube, and ovary.In light of a changed intraoperative finding, the amputation of the left rudimentary horn and prophylactic bilateral salpingectomy were performed. Main outcome measuresShowing the fibroid degeneration and growth of a non-functional, non-communicating rudimentary horn in a unicornuate uterus (ASRM classification 2021) treated laparoscopically. ResultsLaparoscopic removal of the uterine horn was successful, and no intra- and postoperative complications occurred. The patient was in good health at the six-month follow-up visit. The histopathological examination confirmed the fibroid degeneration and the absence of endometrium. ConclusionThe lack of symptomatic cases of rudimentary non-functional horns reported in the literature led gynecologists to consider them a silent Mullerian anomaly. This unique case demonstrates that even non-functional rudimentary horns can undergo symptomatic transformation processes requiring surgery. This information may be helpful for more comprehensive counseling of women and for considering the possibility of this occurrence in the diagnostic work-up of misleading Müllerian anomalies. With this in mind, surgical treatment can also be better planned as the technical aspects change compared to what is expected for an adnexal pathology.