We report a case of Mazabraud syndrome diagnosed in a 53 year old female patient. This disease is characterized by the association of a fibrous dysplasia with one or several intramuscular myxoma(s). The literature related to this pathology is poor and only reports a few cases. The aetiology has not been fully established: a genetic hypothesis seems most likely given the common association with McCune-Albright syndrome. Although rare, the incidence of Mazabraud syndrome seems underestimated, probably out of ignorance. The aim of this article is to describe Mazabraud syndrome and its common features. The management of fibrous dysplasia of bone and benign soft tissue tumors will also be outlined.