The present case highlights the potential for safe postpubertal conservative gonadal management of CAIS.

Serial ultrasound evaluations facilitate the early detection of gonadal abnormalities.

Despite the development of gonadal tumors during puberty, histopathological analysis confirmed benign lesions.

Gonadectomy may be deferred with vigilant monitoring.

The timing of gonadectomy in patients with complete androgen insensitivity syndrome (CAIS) remains controversial due to concerns about the potential risk of malignancy. We report a rare case of CAIS diagnosed shortly after birth, prompted by a discrepancy between the karyotype identified through amniocentesis and the phenotype at birth. Early diagnosis allowed for longitudinal evaluation of the gonads from birth through puberty. Although gonadal tumors developed during puberty, histopathological examination revealed benign lesions. These findings suggest that, with careful monitoring, gonadectomy might have been safely deferred until adulthood in this patient.

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© 2025 The Authors. Published by Elsevier Inc.