Genitoplasty for the management of 46,XX disorders of sex development and congenital adrenal hyperplasia

46,XX disorders of sex development (DSD) resulting from congenital adrenal hyperplasia (CAH) lead to varying degrees of genital virilization and raise complex questions regarding gender assignment, timing of intervention, and long-term outcomes. Feminizing genitoplasty aims to restore functional anatomy, preserve urinary and sexual function, and support psychosocial well-being within a multidisciplinary and ethically informed framework. While many individuals with CAH retrospectively favor childhood surgery, population data show heterogeneous views, supporting a case-by-case approach rather than a universal moratorium. Ethical and legal standards emphasize transparent discussion of risks, alternatives, and long-term outcomes; consent must be qualified, persistent, written, and reaffirmed over time. Early surgery may be beneficial in selected cases-particularly where functional obstruction or significant virilization is present—yet deferral remains appropriate when uncertainty prevails. Contemporary management integrates nerve-sparing techniques, urogenital sinus reconstruction, and long-term psychosocial support, underscoring the need for individualized, evidence-based care for children with CAH-related DSD.