An ovarian juvenile granulosa cell tumor in a 15-year-old white girl is reported. The patient had enchondromatosis (Ollier's disease). amd a review of the literature revealed two previous reports linking enchondromatosis with ovarian sex-cord stromal tumors. This heretofore unrecognized association between these two unusual lesions indirectly supports a generalized mesodermal dysplasia in patients with enchondromatosis. It also draws attention to the possible emergence of ovarian neoplasms in addition to the more frequently encountered chondrosarcomas.