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Hormone secreting juvenile granulosa cell tumor of the ovary in an infant presenting with massive ascites and precocious pseudopuberty

Juvenile granulosa cell tumors (JGCTs), account for 4–5% of GCTs, the rare sex cord stromal tumors. Presentation of JGCTs in infants is even rarer. We present case of a 3-month-old female infant with abdominal distension, feeding intolerance and fever of 10 days' duration. The child had gross abdominal distension causing visible respiratory distress. She also had signs of isosexual precocious puberty. Radiological evaluation was suggestive of left ovarian tumor. Left oophorectomy with preservation of the fallopian tube was done after hemodynamic stabilization. Histopathology evaluation of the tumor was suggestive of JGCT, which was confirmed on immunohistochemical evaluation. On 1 year follow-up, the child is stable, has achieved age-appropriate milestones and is tumor-free. Parents have been explained importance of regular follow-up to detect tumor recurrence. This is probably the youngest case of JGCT presenting with precocious puberty reported in literature so far.