Hyperparathyroidism-jaw-tumor syndrome (HPT-JTS) is a rare autosomal dominant disorder. A typical manifestation of HPT-JTS is the association of jaw-ossifying fibroma with primary hyperparathyroidism. Due to its rarity and diversity in its manifestations, it is a challenging diagnosis. A 33-year-old woman was referred due to painful swelling of the right maxilla suggestive of malignancy. The clinical presentations were not conclusive until she underwent F18-fluorodeoxyglucose positron emission tomography/computed tomography (F18-FDG PET/CT). F18-FDG PET/CT proved to be a useful tool to assist the clinicians in visualizing the "bigger picture" and, therefore all manifestation as pieces of "one puzzle."
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