Ollier's disease (OD) is a rare disorder associated with the presence of multiple enchondromas. Granulosa cell tumors are rare sex cord-stromal ovarian tumors. This is the first report of a patient in her fourth decade with a combination of OD and juvenile granulosa cell tumor.A 36-year-old woman with OD developed an ovarian tumor. The tumor was found at a routine MRI scan. During surgery a stage IIc granulosa cell tumor was removed; pathologic examination showed a juvenile type. A review of literature showed 8 previous cases of (juvenile) granulosa cell tumor associated with OD.The coexistence of granulosa cell tumors in patients with OD is more frequent than expected by chance. We suggest that patients with OD should undergo regular gynecological investigation.