en Obstetrics and Gynecology Obstetrics and Gynecology گزارش مورد Case Report <div style="text-align: justify;"><span style="font-size:10pt"><span style="line-height:121%"><span new="" roman="" style="font-family:" times=""><b>Background: </b>Mature cystic teratomas (dermoid cysts) are common benign ovarian tumors; however, their presentation within an inguinal hernia is exceedingly rare. When accompanied by features of a disorder of sex development (DSD) and coexisting M&uuml;llerian&ndash;renal anomalies, diagnostic evaluation becomes significantly more complex.</span></span></span><br /> <span style="font-size:11pt"><span style="line-height:11.95pt"><span new="" roman="" style="font-family:" times=""><b><span style="font-size:10.0pt">Case Presentation: </span></b><span style="font-size:10.0pt">We report a 30-year-old nulligravida with an eight-year history of primary</span></span></span></span><br /> <span style="font-size:10pt"><span style="line-height:115%"><span new="" roman="" style="font-family:" times="">infertility and a progressively enlarging left groin mass. Physical examination showed short stature, webbed neck, shield chest, and external genital findings consistent with a DSD, including a 3<span cambria="" math="" style="font-family:">‑</span>cm phallus<span cambria="" math="" style="font-family:">‑</span>like structure with a preputial hood. Imaging revealed a unicornuate uterus and a large adnexal cyst herniating into the inguinal canal. Computed Tomography )CT( urogram demonstrated a malrotated, non<span cambria="" math="" style="font-family:">‑</span>functioning left kidney. During laparotomy and inguinal exploration, the hernia sac contained the left ovary, a broad<span cambria="" math="" style="font-family:">‑</span>ligament cyst, and a rudimentary uterine horn. Histopathology confirmed a mature cystic teratoma. Postoperative recovery was uneventful, and the patient received multidisciplinary counseling.</span></span></span><br /> <span style="font-size:10pt"><span style="line-height:121%"><span new="" roman="" style="font-family:" times=""><b>Conclusion: </b>This rare case highlights the need to consider underlying DSD and congenital M&uuml;llerian&ndash;renal anomalies when encountering inguinal adnexal herniation in individuals presenting with infertility or atypical phenotypic features. Accurate anatomical assessment and multidisciplinary management are essential for optimal care.</span></span></span></div> Anomalies, Hernia, Ovary, Uterus 20 23 http://caspjrm.ir/browse.php?a_code=A-12-169-2&slc_lang=en&sid=1 Narayanaswamy Kavitha kavitha.nrnswm@gmail.com 10031947532846002647 0009-0008-3874-207 Yes Postgraduate, Department of Obstetrics and Gynecology, Guntur Medical College and General Hospital, Guntur, Andhra Pradesh, India Potharaju Jayanthi 10031947532846002648 10031947532846002648 No Lavanya Palli 10031947532846002649 10031947532846002649 No