Abstract
BACKGROUND: Müllerian anomalies complicated by extrapelvic endometriosis are uncommon, and herniation of Müllerian remnants into the inguinal canal is exceptionally rare. These complex presentations pose significant diagnostic challenges arising from intricate embryological maldevelopment, necessitating multidisciplinary collaboration to optimize both patient safety and fertility preservation. CASE REPORT: We report a case in which a non-communicating rudimentary uterine horn and endometriotic tissue presented as an incarcerated inguinal hernia in a woman with a solitary pelvic kidney. Multimodal imaging (3D ultrasound, CT with 3D reconstruction, and MRI) enabled preoperative mapping and guided a one-stage laparoscopic approach. Postoperative GnRH-agonist therapy was initiated for adjuvant suppression, and longer-term follow-up is ongoing. CONCLUSION: This case underscores the importance of considering Müllerian anomalies and endometriosis in the differential diagnosis of inguinal masses in women with genitourinary malformations. Multidisciplinary integration of gynecological, urological, radiological, and surgical expertise is essential for accurate diagnosis and safe, fertility-sparing management.