We report a case of 6 years old girl with ovarian Steroid Cell Tumor, Not Otherwise Specific (SCT-NOS). She was admitted to the hospital with increased weight gain, hypertrichosis, and cushingoid facies in form of prominent cheecks, moon facies with dorsocervical fat pad, obese built, acanthosis nigricans on back of neck and marked hirsutism. Initial workup showed raised level if cortisol with loss of diurnal rhythm, and undetectable Plasma ACTH levels, increased testosterone levels. Ultrasound revealed left sided adnexal mass having no flow inside, which was later confirmed on CT as neoplastic lesion of left ovary measuring 6.9 X 8.1 X 4.9 cm. The patient underwent left oophorectomy and pathological examination showed steroid cell tumour, limited to ovary and negative for malignancy. She was kept on IV hydrocortisone preop and post operatively and antihypertensives. After tumor resection her testosterone and cortisol returned to normal levels. Keywords: steroid cell tumor; not otherwise specific; cushing syndrome; ovarian mass.