The fibrous dysplasia of bone is painless, benign, and slowly progressive bone lesion. It may rarely become aggressive by compression of adjacent organs or malignant transformation. This disease falls within the scope of a McCune-Albright syndrome in less than 7 % of cases. The authors reported the case of a 25-year-old woman, living in the countryside, who suffered from a severe craniofacial fibrous dysplasia which has begun since her childhood with no medical care. Her clinical picture was that of McCune-Albright syndrome, and CT scan revealed advanced dysplasia with endocranial compression.