Granulosa cell tumors (GCT) of the ovary represent less than 5% of malignant ovarian tumors. Primary treatment of GCT is surgery. GCT present indolent growth and also tend to relapse many years after diagnosis. Radiotherapy, chemotherapy and hormonal therapy are of little benefit. We report a case of a 60–year–old woman with a heavily pretreated recurrent, c–kit positive, GCT of the ovary who underwent an experimental therapy with imatinib, a tyrosine kinase inhibitor. Imatinib (400 mg/day during the first 2 months; 800 mg/day after) was given, without notable side–effects. Monthly positron emission tomography–computed tomography scan evaluations were performed revealing a marked reduction of disease after 6 months of treatment. To our knowledge this is the first case of highly recurrent and unresponsive GCT of the ovary responding to imatinib. Further studies evaluating this drug in recurrent and/or aggressive GCT are warranted.