Background: Granulosa cell tumor (GCT) is an uncommon malignant neoplasm primarily arises from the sex–cord stromal cells of the ovary. They constitute only 2% of the ovarian tumors and more than 70 % of the sex cord–stromal tumors. There are two distinct histological types: Juvenile GCT represents only 5% of this tumor and usually occurs in pre–pubertal girls and women younger than 30 years whereas Adult GCTs present with early stage disease and are commonly encountered in the age group of 35 years to 55 years. These tumors have a particular clinical and histological profile, and may reoccur up to 40 years after diagnosis in 25% cases.

Case report: A 22 year old female patient, nulligravida, with 4 years of active married life, presented to gynaecology oncology clinic with lower abdominal pain, and distension and breathlessness after mild work since 1 month.. On examination, she had Pulse Rate of 96/min and her blood pressure was recorded as 110/70 mm Hg, pallor was present,  on per abdominal examination, ascites was present ,mass was felt arising from pelvis extending above umbilicus and was non tender. On local examination clitoromegaly was present. Vaginal examination revealed healthy cervix and vagina and normal sized uterus and mass felt as described above in right adnexal region. All blood investigations including tumor markers germ cell tumor markers were within normal limits except for the CA–125 which was 900 U/ml and hormonal profile was normal. Ascitic fluid and pleural fluid tapping was done and approx 600 ml of pleural fluid drained out and sent for examination and showed no malignant cells in fluids. Chest radiograph showed hydro–pneumothorax and thus intercostals drainage tube was kept. With provisional diagnosis of ovarian neoplasm she was taken up for laparotomy. Final histopathological examination report showed juvenile granulosa cell tumor with intact capsule. So patient was staged 1A of ovarian neoplasm. She is on chemotherapy at present. 

Conclusion: This was a rare case of juvenile granulosa cell tumor that presented with abdominal pain along with breathlessness and ascites and pleural effusion resembling germ cell type picture.

It turned out to be juvenile granulosa cell tumor with unusual presentation. Since they are less frequent and may present with unusual clinical picture, their differential cannot be overlooked in any age group.This patient carries good prognosis and 5 year survival of 95%. She is suggested for child bearing 6 months after completion of chemotherapy.