Ovarian granulosa cell tumors (GCTs) are uncommon neoplasms that arise from the sex-cord stromal cells of the ovary. GCTs are characterized by long natural history and their tendency to recur years after the initial diagnosis. They present with symptoms and signs due to estradiol secretion, including vaginal bleeding and precocious puberty. Occasionally, tumor rupture causes abdominal pain and hemoperitoneum. GCT is usually associated with a mass on pelvic examination which is subsequently confirmed with imagine techniques. Surgery is the mainstay of initial management for histological diagnosis, appropriate staging and debulking surgery. In patients with stage I disease and those in reproductive age a more conservative unilateral salpingo-oophorectomy is indicated. In postmenopausal women and those with more advanced disease a total abdominal hysterectomy with bilateral salpingo-oophorectomy is the appropriate surgical treatment. The most important prognostic factor associated with a higher risk of relapse is the stage of disease. The role of post-operative chemo- or radiotherapy in stage I disease and those with completely resected tumor has not been defined. Nevertheless, the use of adjuvant chemotherapy or radiotherapy has sometimes been associated with prolonged disease-free survival and possibly overall survival. Chemotherapy should be considered for patients with advanced, recurrent or metastatic disease and the BEP (bleomycin, etoposide, cisplatin) is the currently preferable regimen. Although overall response rate (RR) is high, the impact on disease-free or overall survival is unknown. Due to their tendency to recur years after the initial diagnosis, prolonged surveillance is essential.