OBJECTIVE: To present a rare case of multiple pituitary adenomas with a functional follicle-stimulating hormone component leading to ovarian hyperstimulation syndrome. METHODS: We present the clinical, laboratory, imaging, and pathologic findings along with a review of the literature. RESULTS: A 28-year-old female presented with 5 months of amenorrhea and abdominal pain. Physical exam was unremarkable. Labs revealed elevated prolactin (94 ng/mL), elevated estradiol (608 pg/mL), inappropriately normal follicle-stimulating hormone (10.2 mIU/mL), and suppressed luteinizing hormone (0.36 mIU/mL). Transvaginal ultrasound showed numerous large ovarian cysts bilaterally. Brain magnetic resonance imaging showed a 1.2-cm sellar mass. Various medical therapies were not tolerated due to side effects and the patient underwent gross total resection of the sellar mass with marked improvement in her symptoms and blood hormone levels, resumption of menstruation, and shrinkage of the ovarian cysts. Histologic examination revealed 3 separate staining patterns consistent with multiple pituitary adenomas. CONCLUSION: Functioning gonadotroph adenomas are rare and often difficult to diagnose, though in premenopausal women they can lead to the distinct clinical presentation of spontaneous ovarian hyperstimulation syndrome. The favored treatment approach is surgical as it has the highest reported success rate. Recurrence is not uncommon and long-term surveillance is recommended. Given limited data on long-term follow-up, the role of available therapies is not well defined, and further research is needed. To our knowledge, this is the first reported case of multiple pituitary adenomas that included a functional gonadotroph component.