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Steroid cell tumours of the ovary are uncommon sex-hormone-secreting tumours characterized by steroid cell proliferation. The incidence of steroid cell tumour of the ovary is only 0.5% of all ovarian tumours. Here, we present a case of a 59-year-old post-hysterectomy patient who presented with complaints of excessive hair growth, hoarseness of voice and male pattern baldness for 1 year. Transvaginal ultrasonography revealed a well-defined solid 4 × 3 cm hypoechoic lesion, and there was increased heterogeneous vascularity on colour doppler suggestive of neoplastic solid mass lesion of the right ovary. A blood examination showed total serum testosterone of 76 ng/dL, DHEAS—310 ug/dL, and 17-OH progesterone—1025 ng/dL. Her haemoglobin in the preoperative period was 18 gm/dL, though erythropoietin levels were normal. The patient was found positive for JAK2 gene mutation, which is strongly associated with polycythaemia vera. Laparotomy followed by bilateral salpingo-oophorectomy and frozen section was planned. Since frozen section was positive for malignancy in right ovarian mass, omentectomy, bilateral pelvic and para-aortic lymphadenectomy with multiple peritoneal biopsies were performed. The result of histopathology confirmed the diagnosis of steroid cell tumour, probably Leydig cell tumour. A blood examination after a month revealed a marked decline in serum testosterone, i.e. 4.9 ng/dL, DHEAS—15.3 ug/dL, and 17-OH progesterone—0.13 ng/dL.