Abstract Granulosa cell tumors are uncommon neoplasms that are characterized by their long natural history and for their tendency to recur years after an apparent clinical cure. As such they are difficult tumors to study. In the majority of cases, the initial therapy is surgical although the extent of the necessary procedure remains controversial. The role for any adjuvant therapy after complete resection remains to be established. In patients with advanced disease, combination chemotherapy consisting of cisplatinum/vinblastine/bleomycin has the highest identified response rates. In patients with recurrent or metastatic disease therapy is palliative and has not yet been standardized. Treatments therefore need to be individualized.