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Preterm ovarian hyperstimulation syndrome (POHS) is characterized by swelling of the vulva and hypogastrium, elevated estradiol and gonadotropin levels, and multiple ovarian follicular cysts in premature baby girls. We report a case of POHS with a review of the literature. The patient presented with swelling of the clitoral hood, and the labia majora and minora. We confirmed multiple ovarian cysts, as well as elevated estradiol and lutenizing hormone levels by performing pelvic ultrasound and laboratory tests. The symptoms resolved gradually and spontaneously.