Ovarian tumors and cysts are rarely seen in children. They constitute 1-2% of solid tumors among children. Over tumors are categorized into three main groups: epithelial, embrionic, and sex cord-stromal tumors. Sex cord-stromal tumors arise from primordial cell cords that are located in the center of the ovary during embriological development. We have detected a patient who was diagnosed with a juvenile granulosa cell tumor (JGCT) at two years of age after presenting with excessive breast enlargement during the past month. She had a large mass in the abdomen, ascites, and marked pleural effusion in the right hemithorax. This association was Pseudo-Meigs syndrome. The association of JGCT and Pseudo-Meigs syndrome is a considerably rare event. Moreover, since the patient was the youngest among all previously reported cases in existing literature, our observations indicate that a juvenile granulosa cell tumor may occur at an early age.In conclusion, Pseudo-Meigs syndrome with JGCT shouldn't forget, even in young children.