RESUMO
Mazabraud's syndrome is characterized by myxomas of intramuscular type present in association with fibrous dysplasia. Up to this day, approximately 80 cases of Mazabraud's syndrome have been reported, although in the head and neck territory intramuscular myxoma reports in association with fibrous dysplasia of the bone are very scarce. An unusual case of Mazabraud's syndrome in a 63 years old female displaying fibrous dysplasia of the mandible and soft tissue myxoma in the edentulous alveolar ridge in the molar area is reported. After four years of follow-up, the clinical, imagenological and microscopical findings that led to the diagnosis and treatment are discussed. This report exemplifies the diagnostic and treatment challenge of this rare disease and enhances our clinical knowledge due to its long follow-up, highlighting the need of understanding better its behavior in order to establish proper guidelines for its treatment.
