Objective To analyze the clinical data of patients with ovarian juvenile granulosa cell tumor (JGCT) with ascites as the first symptom, and to review the relevant literatures, and to provide reference for clinical diagnosis. Methods The clinical manifestations, ascites properties, imaging and laparoscopic ovarian tumor features, treatment methods and prognosis of ovarian JGCT patients with ascites as the first symptom were retrospectively analyzed and discussed with the literature review. Results A 19-year old girl showed an increase in progressive abdominal distension accompanied by anorexia discomfort and outpatient B ultrasound prompted a large number of pelvic pelvic fluid. The ascites nature of the patient was the effusion of lymphocyte elevation. Ascites tumor and serum CA125 Significantly increased. Abdominal CT showed left attachment area teratoma, right attachment area capsule solid change? Laparoscopic exploration confirmed that the patient's right ovarian pathology immunohistochemistry was juvenile granulosa cell tumor, stage for the IC period. Patients underwent TP regimen chemotherapy, followed up for 6 months to date without recurrence. Conclusion Taken together, we should be highly vigilant the juvenile granulosa cell tumor with massive ascites as the first clinical manifestation.