Purpose of reviewGranulosa cell tumours of the ovary are an uncommon ovarian sex-cord stromal tumour. These neoplasms provide a spectrum of clinical presentations that span from the first to the tenth decade. Surgery represents the primary therapy for early stage disease; however, management of women with advanced disease is less clear. Because of their relative rarity, evidence to support decision-making in the management of granulosa cell tumours is limited. The purpose of this review is to provide the clinician with an updated knowledge of the clinical and molecular aspects of granulosa cell tumours in order to guide therapy.Recent findingsThe clinical stage, mitotic index and cellular atypia correlate most strongly with prognosis. However, these tumours may demonstrate heterogeneous genetic aberrations that can predict behaviour and response to therapy. Case series and reports suggest that postoperative combination chemotherapy is of most benefit in advanced disease. Serial measurements of serum inhibin may be helpful in the follow-up of these women, particularly in the post-menopausal group.SummaryThe pathology and treatment of women with granulosa cell tumours of the ovary is complex. Such women should be managed in a multidisciplinary gynaecological oncology unit. A better understanding of the molecular pathology may assist treatment.