• Sundar Shrestha, Niroj Banepali, Rakesh Sthapit, and Dipesh Agrawal.
• Department of General Surgery, Bir hospital, Kathmandu, Nepal.
• JNMA J Nepal Med Assoc. 2019 Mar 1; 57 (216): 127-129.

AbstractThere are various causes of primary amenorrhea in phenotypically females such as, complete androgen insensitivity syndrome, pure gonadal dysgenesis, 17b-hydroxysteroid dehydrogenase deficiency, or mixed gonadal dysgenesis. Primary amenorrhea in a phenotypically female is commonly encountered in Androgen Insensitivity Syndrome. In patients with Androgen Insensitivity Syndrome, with intra-abdominal testis there is high chances of developing testicular tumour, among them Sertoli cell tumour and seminoma being the most common types. Leydig cell tumour in androgen insensitivity syndrome, is very rare and malignant leydig cell tumour is even further rarer. There are few cases reported in the literature of malignant leydig cell tumour with complete androgen insensitivity. Here we are reporting a case of 65 years married elderly patient with malignant leydig cell tumour with complete androgen insentivity syndrome. Keywords: complete androgen insensitivity syndrome; leydig cell tumour; testicular feminization.

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