We present a rare clinical report of a 45-year-old man affected by Systemic Lupus Erythematosus (SLE) with a unilateral mass of the left frontal bone, diagnosed as a possible recurrence of fibrous dysplasia. This patient was evaluated with computed tomography (CT scan) and was treated with resection of the mass and reconstruction with splitting a calvarian bone graft. The pathological evidence was suggestive for a bone cavernous haemangioma. No previously described cases of bone dysplasia associated with this systemic syndrome were reported so far. Patient's disease was under stringent control at the time of hospitalization, and the outcome has been successful, even though a mild increase of inflammatory indexes was reported after surgery. This laboratory evidence was transient and not related to further clinical complications.