FOXL2 mutation is diagnostic, and, if pathology is in doubt, it should be performed.• Surgical resection and staging is key to assess prognosis.• Platinum-based adjuvant chemotherapy such as with carboplatin plus paclitaxel is a reasonable step for women diagnosed with distant recurrences or poor prognostic initial features. However, evidence for improved outcome is lacking. • Hormone-based therapies, especially with aromatase inhibitors, have shown activity and future studies may lead to further insight on the relationship of inhibition of hormonal pathways and tumor progression.Adult-typegranulosa cell tumors (GCTs), although rare, are the most commonly diagnosed neoplasms arising in the endocrine-active ovarian stroma. They are characterized by excessive production of estrogens, antimullerian hormone and inhibins. In 2009, a specific mutation in FOXL2 was identified to be pathognomonic of GCTs. How dysregulation of this transcription factor, resulting in upregulation of aromatase, leads to unchecked proliferation, and progression to a malignancy, remains unclear. The key pathological and clinical feature of GCTs that affects their usually favorable outcomes is a diagnosis of greater than Stage 1 disease at presentation. Chemotherapy is given as adjuvant upon an advanced stage diagnosis; however, its effect on survival upon recurrence is modest. On the other hand, aromatase inhibitors also lead to tumor regression and are suitable for long-term maintenance.