Articles

<b>Background:</b> Morris syndrome, also known as androgen insensitivity syndrome (AIS), is a disorder of sex development caused by mutations in the androgen receptor gene in individuals with a 46,XY karyotype. The syndrome is typically diagnosed during adolescence due to primary amenorrhea, and early detection in childhood is uncommon. Prompt recognition is clinically important to guide multidisciplinary management and reduce the risk of gonadal malignancy.<b> Case Description:</b> We report the case of a one-year-old phenotypic female presenting with an irreducible, painful right inguinal hernia. Ultrasound suggested bilateral inguinal hernias containing ovary-like structures. During emergency surgical exploration, bilateral testes were unexpectedly identified within the inguinal canals. The testes were reduced into the abdominal cavity, and herniotomy with anterior wall repair was performed bilaterally. External genital examination revealed normally developed labia but absence of the hymenal ring and vaginal opening. The postoperative course was uneventful. Despite referral for karyotype analysis and multidisciplinary follow-up, the parents did not return for further evaluation. <b>Conclusion:</b> This case highlights the rare intraoperative diagnosis of Morris syndrome in early childhood. Awareness of AIS is essential for pediatric surgeons managing inguinal hernias in phenotypic females. Early detection allows timely planning of gonadectomy and long-term multidisciplinary management, minimizing the risk of malignant transformation of undescended testes and optimizing patient outcomes.