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Juvenile granulosa cell tumor is a rare tumor diagnosed in children, which can present with precocious puberty. We have reported a case of a 6-month-old female patient who presented with precocious puberty. Abdominopelvic imaging revealed a large mixed cystic and solid mass, with internal solid enhancement, and restricted diffusion. At surgery, mass was confirmed to arise from the left ovary, and histopathology confirmed juvenile granulosa cell tumor. We provide a literature review of juvenile granulosa cell tumor and discuss imaging characteristics of this diagnosis.