Background <p>Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterized by Müllerian duct anomalies. Patients have a significantly higher incidence of inguinal hernia (6.4%-15.6%) compared to the general female population. These hernias frequently contain ovarian or tubal tissue and are associated with a high risk of strangulation, posing a threat to future fertility. This case report details a rare pediatric presentation of MRKH syndrome type II with a strangulated inguinal ovarian-tubal hernia.</p> Case presentation <p>A 7-year-5-month-old girl presented with a 28-hour history of an irreducible left inguinal mass. Ultrasound revealed a left incarcerated inguinal hernia and a right ectopic kidney. Emergency surgery confirmed a left strangulated inguinal hernia containing a necrotic ovary and fallopian tube, along with a contralateral occult hernia. Findings of uterine and vaginal agenesis with a 46,XX karyotype confirmed MRKH syndrome type II. A literature review of 24 cases (including ours) showed that inguinal hernias in MRKH are predominantly left-sided (45.8%) or bilateral (41.7%), with ovarian involvement in 87.5% of cases. In type II MRKH, renal malformations were observed in 91.7% of cases and were consistently associated with an ipsilateral inguinal hernia.</p> Conclusion <p>Inguinal hernias in MRKH syndrome commonly involve gonadal tissue and carry a high incarceration risk. The presence of reproductive organs within a hernia sac or associated renal anomalies should raise strong suspicion for MRKH syndrome. Early diagnosis, timely surgical intervention to preserve viable ovarian tissue, and multidisciplinary management are crucial for protecting reproductive potential and improving long-term outcomes.</p>