Introduction and importance: Disorders of sexual development (DSD) are a rare but disastrous condition. A plethora of biological, psychological, and ethical problems surround the management of DSD cases. Case presentation: A case of 25-year-old woman with a complaint of primary amenorrhea was presented. She wanted to be examined as she had planned to be married. However, examinations revealed that she had 46, XY karyotype, complete androgen insensitivity syndrome, and vaginal agenesis. After several family meetings, the patient, her family, and her partner decided that she would remain as a woman despite the results. Clinical discussion: Disorders of sexual development are not only about the biological complexities, but also the psychological, social, and ethical dilemmas faced by both the patient and clinicians. A coordinated care team plays a crucial role in addressing the unique challenges presented by DSD, particularly for those diagnosed later in life. Conclusion: Comprehensive and multidisciplinary approach is essential for managing DSD patients.
- Ambiguous genitalia
- Disorders of sexual development
- Gender assignment
- Social stigma