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Mazabraud's syndrome is a rare disorder, the main characteristics of which are fibrous dysplasia of bone associated with intramuscular myxomas. The metabolic characteristics of intramuscular myxomas, associated with fibrous dysplasia, have not previously been described with 18F-FDG-PET. Our case demonstrates that there is low, but not insignificant uptake associated with these intramuscular myxomas, with a standardized uptake value (SUV) range between 1.3 and 2.6. As such, this entity merits consideration when evaluating hypoattenuating intramuscular masses, particularly in the context of fibrous dysplasia.