Partial Androgen Insensitivity Syndrome (PAIS), a 46XY disorder of sexual differentiation where there is partial loss of function of androgen receptors (AR). The spectrum of clinical presentation can vary in adult from phenotypic female with mild virilization to under virilized male with or without genital ambiguity. Case description: A 25 years old woman presents with primary amenorrhea, ambiguous genitalia and features of mild virilization like hirsutism and voice change. After admission in Reproductive Endocrinology and Infertility Department of Bangabandhu Sheikh Mujib Medical University, she underwent genitoplasty, vestibuloplasty and bilateral gonadectomy. Histopathology of the atrophic testes revealed Sertoli cell tumor. Conclusion: Partial Androgen Insensitivity Syndrome (PAIS) is presented clinically as ambiguous genitalia, as predominant female to male phenotype with varying degree of virilization. Multidisciplinary approach of management aims at appropriate sex assignment, reconstructive surgery, gonadectomy to prevent tumor genesis and hormone replacement therapy.