Urethral strictures in virilized 46,XX males are rare and complex to manage.

Only three studies reported relevant cases, with limited surgical outcome data.

One documented buccal graft urethroplasty showed successful short-term results.

Open or robotic reconstruction is preferred for complex or obliterative strictures.

More case reports and long-term data are needed to guide treatment protocols.

Disorders of sex development in 46,XX male individuals are rare and present complex surgical challenges, particularly regarding urethral stricture following urethral surgery. We present a 46,XX individual raised male who underwent early urethral surgery and developed a urethral stricture in adulthood. Surgical repair was not feasible due to anatomy and comorbidities. A systematic review on the management of urethral strictures in this patient population was conducted. Only one case of successful buccal mucosa graft urethroplasty in a 46,XX male was found. This case underscores the scarcity of data and challenges in managing urethral strictures in virilized 46,XX individuals.

46

XX male

Urogenital sinus

Urethral stricture

Urethral surgery

© 2025 The Authors. Published by Elsevier Inc.