Juvenile granulosa cell tumor is a rare gynecologic malignancy. We describe a case of this tumor arising in a young woman. Initially assigned to Stage IC, the patient returned 3 months later with metastatic disease. Imaging findings in this case are discussed and pathologic examination confirms the diagnosis. The epidemiology, natural history, presentation, histologic and imaging appearances, prognosis and treatment of this malignancy are reviewed.

Abbreviations: CT, computed tomography; MRI, magnetic resonance imaging