Granulosa cell tumours account for only 5% of ovarian malignancies and there is currently no standard treatment. It is considered as a low-grade malignancy with a favorable prognosis but relapse and extraovarian spread may occur as late as 20 years after diagnosis. It is thus important to better define the characteristics and prognostic factors of this histological subtype in order to recommend appropriate therapy. We performed a review of the literature. This disease is most commonly diagnosed in patients around 50 years old or at puberty. In most cases patients present identical symptoms associated with endometrial hyperplasia or neoplasia. Call-Exner bodies and "coffee bean" cells are characteristic of the histopathology of these tumors. Various prognostic factors are currently being evaluated. Surgery is the treatment for local disease. Patients with locally advanced, recurrent or metastatic tumors require chemotherapy, although the optimal regimen remains to be determined.