Craniofacial fibrous dysplasia is a rare sub-type of fibrous dysplasia which is a congenital, noninherited, benign intramedullary bone lesion in which the normal bone marrow is replaced by abnormal fibro-osseous tissue of the skull and facial bones. The clinical manifestation of cranial asymmetry, facial deformity, fractures or other abnormalities necessitates a comprehensive approach for accurate diagnosis and optimal management. We present the case of a 12-year-old female who presented with one-year history of progressive facial and skull swellings, visual impairment and headache.

General physical examination revealed swelling of the skull, especially the left frontoparietal region. Laboratory tests were unremarkable except for the elevated levels of alkaline phosphatase and phosphorus. A brain computed tomography showed expansile lesions with ground-glass changes in the left frontal bone, parietal bone and clivus.

A diagnosis of fibrous dysplasia was made which was confirmed by Bone biopsy and histology. This case underscores the importance of collaboration between Radiologists, Neurosurgeons and Pathologists in achieving an accurate diagnosis and satisfactory outcome for craniofacial fibrous dysplasias which are rare tumours.