Objective: To discuss clinical and pathological characteristics of the ovarian juvenile granulosa cell tumor. Methods: 1 case in our hospital with suspected juvenile granulosa cell tumor of scleroring stromal tumor was reported and literatures were reviewed. Results: Female, 19-year-old, lower abdominal pain more than 2 months, B ultrasound, MRI and tumor markers all suggested that it was the benign ovarian tumor. An ovarian tumor was found in the right side of ovary and the size is 4 cm × 3 cm × 2 cm. After cutting the surface, it was typically solid with cysts formed. The histopathological changes displayed solid flakes, nodular arrangement. The tumor cells were round or oral, cytoplasm stained pink. Nuclear grooves were occasionally conspicuous and mitosis figures could be found. Some areas tumor cells were luteinized obvious. The immunohistochemical revealed that inhibin-α, CD99, Vimentin, S-100, SMA were all positive. We followed up the patient for 12 months, menstruation was returned to normal and no tumor recurrence. Conclusion: Ovarian juvenile granulosa cell tumor is a rare tumor and researcher should pay enough attention to its clinical pathological features.