Steroid cell tumour is a rare sex cord-stromal tumor of the ovary. It may produce steroids and is associated with testosterone secretion which causes symptoms like hair loss, hirsutism, and oligomenorrhea/amenorrhea due to hormonal activity and virilizing properties of tumor. In this article, we reported a 27-year-old woman who presented with hirsutism, hoarseness of voice, scalp hair fall, and amenorrhea for 8 years. Clinical and diagnostic evaluation revealed a left adnexal mass and elevated serum levels of testosterone and she was diagnosed as having a Sertoli Leydig cell tumour of ovary. She underwent left salpingooophorectomy and both histopathological examination and immunohistochemistry confirmed the diagnosis. Her serum testosterone levels normalized 3 days after the surgery and her menses resumed spontaneously a few months after the operation. In addition, we reviewed the literature on the epidemiology, clinical presentations, imaging and histological findings, and the treatment options on this disease.