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Background Multiple Endocrine Neoplasia Type 1 (MEN1) is a rare autosomal dominant disease that generally presents with primary hyperparathyroidism. However, initial presentation may vary and continued reevaluation of etiology of symptoms is required for appropriate diagnosis. Case Presentation Twelve year old female presented with altered mental status that self-resolved and hypoglycemia. Laboratory evaluation revealed pituitary dysfunction with central hypothyroidism and adrenal insufficiency in the setting of hyperprolactinemia. Macroadenoma was confirmed on imaging. Despite medical treatment of pituitary hormone disorders, she continued to have significant hypoglycemia and further workup revealed hyperinsulinism. Insulinoma was identified and confirmed by endoscopic ultrasound. Hypoglycemia resolved after laproscopic enucleation of the insulinoma. Conclusion Children presenting with one endocrine tumor should be investigated for other potential endocrine tumors. Multiple imaging modalities may be required to confidently identify neuroendocrine tumors for appropriate surgical intervention.