Chronic Obstructive Uropathy Complicating Complete Androgen Insensitivity Syndrome: A Case Report

Complete androgen insensitivity syndrome (CAIS) is a rare X‐linked difference of sex development (DSD) caused by pathogenic variants in the androgen receptor (AR) gene, leading to complete androgen resistance. Diagnosis is often delayed in low‐resource settings due to a low index of suspicion and lack of access to diagnostic testing. We present a 27‐year‐old Ethiopian woman with primary amenorrhea, sparse pubic and axillary hair, small breasts, and a longstanding left inguinal swelling. Uniquely, her clinical course was complicated by chronic obstructive uropathy due to recurrent nephrolithiasis leading to left nephrectomy and severe hydronephrosis in a solitary right kidney—an unusual and under‐reported association. Imaging confirmed the absence of Müllerian structures and the presence of bilateral undescended testes. Hormonal testing showed male‐range serum testosterone (10.81 ng/mL) and karyotype confirmed 46, XY. She underwent laparoscopic bilateral orchidectomy resulting in a significant reduction in serum testosterone and was started on estrogen replacement. This case highlights the importance of early detection of CAIS, comprehensive multidisciplinary care, and awareness of potential urological complications. Additionally, it underscores the psychosocial burden of delayed diagnosis necessitating integrated psychological support to improve patient outcomes.