A 21yrs old female came to endocrine department of Bangabandhu Sheikh Mujib Medical University with complained of hirsutism with male body habitus, male pattern voice since her 12yrs of age. She had primary amenorrehoea. She was sent to National Institute of Nuclear Medicine & Allied Sciences for ultrasonograhy (USG) of whole abdomen. A big complex mass was detected at superoposterior aspect of her left kidney with small sized uterus (AP-1.1cm, L- 3.8cm) and very small ovaries (streak ovary). Preoperative evaluation showed serum dehydroepiandrosterone (DHEA sulphate level >1000µg/dl (1.3 - 9.8ng/dl), testosterone was 451ng/dl (63-120ng/dl), cortisol level was 340.8 nmol/L (138 - 690 nmol/L), 24hrs urinary vanillylmandelic acid (VMA) 13.06 ng/day, Keryotyping showed 46XX. Patient underwent laparoscopic left adrenalectomy; the mass was removed from abdomen. Histopathology report showed adrenocortical neoplasm. Postoperative evaluation was done 4 months later. Report showed that serum DHEA 12.20 ng/dl, serum testosterone level 17.9ng/dl, serum cortisol 705 nmol/L, 24 hrs urinary VMA 7.08ng/day. USG of whole abdomen showed uterus ( AP-2.3cm, L-5.8cm) and ovaries become normal, kidneys were normal, other abdominal organs showed normal findings. Patient was treated by estrogen 0.6mg once daily for 21 days, Medroxyprogesterone tablet 10 mg for 7 days these drugs were continuing for 6 months. Tab spironolactone (50mg) once daily at same duration. Testosterone was again done 1yr after adrenalectomy it was 88.2 ng/dl, Serum cortisol 199 nmol/L. Patient gonodatrophine and sex hormones level was normal. She was conceived 4yrs after this treatment but miscarriage occurred. Her hirsutism was completely cured. Now she was under the treatment of the gynecologist. In ending a successful normalization of reproductive organs as well as rapid decreased of rising hormones level was monitored in this patient.