The purpose of this article is to report a case of ossifying fibroma in the mandible associated with hyperparathyroid-jaw tumor syndrome, in a 46-year-old Caucasian female, surgically resected and reconstructed with iliac crest bone, followed by implant placement. This is a rare syndrome with an autosomal dominant pattern, with the development of primary hyperparathyroidism mainly due to parathyroid adenomas. Fibro-osseous lesions in the jaws can be present and can also precede the development of the endocrine disorder. As renal abnormalities and uterine tumors can develop, an interdisciplinary approach is imperative for its diagnosis and management, due to the possibility of recurrence and potential for malignancy.83 228233