Design: Narrated surgical presentation

Setting: Androgen insensitivity syndrome (AIS) is an X-linked genetic disorder characterized by peripheral androgen resistance due to androgen receptor mutations in subjects with 46 XY karyotype. Despite normal testicular development, the testes are often retained in the abdomen or inguinal canal. Gonadectomy is usually recommended because undescended testicular tissue presents increased risk of malignant transformation after puberty.

Patients or Participants: Video footage and operative photos were used with obtained consent from one patient undergoing laparoscopic bilateral gonadectomy.

Interventions: The patient underwent laparoscopic bilateral gonadectomy.

Measurements and Main Results: N/A

Conclusion: AIS is rare genetic disorder in which androgen receptor mutation leads to female secondary sex characteristics despite testes formation. For complete AIS, gonadectomy is recommended post-puberty to decrease risk of malignant transformation of testicular tissue.